Doege-Potter syndrome
Encyclopedia
Doege-Potter syndrome is a paraneoplastic syndrome
in which hypoglycemia
is associated with the presence of one or more non-islet
fibrous tumor
s in the pleural cavity
. The hypoglycemia is the result of the tumors producing insulin-like growth factor 2
. The syndrome was first described in 1930, by Doege and Potter working independently; the full term "Doege-Potter syndrome" was infrequently used until the publication of a 2000 article using the eponym
.
DPS is rare (as of 1976, less than one hundred cases were described), with a malignancy
rate of 12-15%. Actual rates of hypoglycemia associated with a fibrous tumor are quite rare (a 1981 study of 360 solitary fibrous tumors of the lungs found that only 4% caused hypoglycemia), and are linked to large tumours with high rates of mitosis
. Removal of the tumor will normally resolve the symptoms.
Tumors causing DPS tend to be quite large; in one case a 3 kg (6.6 lb), 23 x mass was removed, sufficiently large to cause a collapsed lung
. In X-ray
s, they appear as a single mass with visible, defined borders, appearing at the edges of the lung
s or a fissure dividing the lobes
of the lungs. Similar hypoglycemic effects have been related to mesenchymal
tumors.
Paraneoplastic syndrome
A paraneoplastic syndrome is a disease or symptom that is the consequence of the presence of cancer in the body, but is not due to the local presence of cancer cells. These phenomena are mediated by humoral factors excreted by tumor cells or by an immune response against the tumor...
in which hypoglycemia
Hypoglycemia
Hypoglycemia or hypoglycæmia is the medical term for a state produced by a lower than normal level of blood glucose. The term literally means "under-sweet blood"...
is associated with the presence of one or more non-islet
Islets of Langerhans
The islets of Langerhans are the regions of the pancreas that contain its endocrine cells. Discovered in 1869 by German pathological anatomist Paul Langerhans at the age of 22, the islets of Langerhans constitute approximately 1 to 2% of the mass of the pancreas...
fibrous tumor
Tumor
A tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer...
s in the pleural cavity
Pleural cavity
In human anatomy, the pleural cavity is the potential space between the two pleura of the lungs. The pleura is a serous membrane which folds back onto itself to form a two-layered, membrane structure. The thin space between the two pleural layers is known as the pleural cavity; it normally...
. The hypoglycemia is the result of the tumors producing insulin-like growth factor 2
Insulin-like growth factor 2
Insulin-like growth factor 2 is one of three protein hormones that share structural similarity to insulin.-Gene structure:In humans, the IGF2 gene is located on chromosome 11p15.5, a region which contains numerous imprinted genes. In mice this homologous region is found at distal chromosome 7...
. The syndrome was first described in 1930, by Doege and Potter working independently; the full term "Doege-Potter syndrome" was infrequently used until the publication of a 2000 article using the eponym
Eponym
An eponym is the name of a person or thing, whether real or fictitious, after which a particular place, tribe, era, discovery, or other item is named or thought to be named...
.
DPS is rare (as of 1976, less than one hundred cases were described), with a malignancy
Cancer
Cancer , known medically as a malignant neoplasm, is a large group of different diseases, all involving unregulated cell growth. In cancer, cells divide and grow uncontrollably, forming malignant tumors, and invade nearby parts of the body. The cancer may also spread to more distant parts of the...
rate of 12-15%. Actual rates of hypoglycemia associated with a fibrous tumor are quite rare (a 1981 study of 360 solitary fibrous tumors of the lungs found that only 4% caused hypoglycemia), and are linked to large tumours with high rates of mitosis
Mitosis
Mitosis is the process by which a eukaryotic cell separates the chromosomes in its cell nucleus into two identical sets, in two separate nuclei. It is generally followed immediately by cytokinesis, which divides the nuclei, cytoplasm, organelles and cell membrane into two cells containing roughly...
. Removal of the tumor will normally resolve the symptoms.
Tumors causing DPS tend to be quite large; in one case a 3 kg (6.6 lb), 23 x mass was removed, sufficiently large to cause a collapsed lung
Pneumothorax
Pneumothorax is a collection of air or gas in the pleural cavity of the chest between the lung and the chest wall. It may occur spontaneously in people without chronic lung conditions as well as in those with lung disease , and many pneumothoraces occur after physical trauma to the chest, blast...
. In X-ray
X-ray
X-radiation is a form of electromagnetic radiation. X-rays have a wavelength in the range of 0.01 to 10 nanometers, corresponding to frequencies in the range 30 petahertz to 30 exahertz and energies in the range 120 eV to 120 keV. They are shorter in wavelength than UV rays and longer than gamma...
s, they appear as a single mass with visible, defined borders, appearing at the edges of the lung
Lung
The lung is the essential respiration organ in many air-breathing animals, including most tetrapods, a few fish and a few snails. In mammals and the more complex life forms, the two lungs are located near the backbone on either side of the heart...
s or a fissure dividing the lobes
Lobe (anatomy)
In anatomy, a lobe is a clear anatomical division or extension that can be determined without the use of a microscope This is in contrast to a lobule, which is a clear division only visible histologically....
of the lungs. Similar hypoglycemic effects have been related to mesenchymal
Mesenchymal stem cell
Mesenchymal stem cells, or MSCs, are multipotent stem cells that can differentiate into a variety of cell types, including: osteoblasts , chondrocytes and adipocytes...
tumors.