Uterine papillary serous carcinoma
Encyclopedia
Uterine serous carcinoma (USC), also known as uterine papillary serous carcinoma (UPSC) and uterine serous adenocarcinoma, is an uncommon form of endometrial cancer
that typically arises in postmenopausal women.
It is typically diagnosed on endometrial biopsy
, prompted by post-menopausal bleeding.
Unlike the more common low-grade endometrioid endometrial adenocarcinoma, USC does not develop from endometrial hyperplasia
and is not hormone-sensitive. It arises in the setting of endometrial atrophy
and is classified as a type II endometrial cancer.
. Such bleeding is followed by further evaluation leading to a tissue diagnosis, usually done by a dilatation and curettage (D&C). A work-up to follow would look for metastasis using imaging technology including sonography and MRI. The median age at diagnosis in a study of 138 women was 67 years, of these 54 had stage I, 20 stage II, 41 stage III, and 23 stage IV disease.
Histopathologically, uterine serous carcinomas is typically characterized by (1) nipple-shaped structures (papillae) with fibrovascular cores (2) marked nuclear atypia (irregularies in the nuclear membrane, enlarged nuclear size), (3) psammoma bodies and (4) cilia.
cancer staging
system.
In 2009, the journal of "Gynecologic Oncology" reported the following 5-year survival rates based upon stage of cancer:
is done at the time of surgery which consists of peritoneal cytology, total hysterectomy
, bilateral salpingo-oophorectomy, pelvic/para-aortic lymphadenectomy
, and omentectomy. The tumor is aggressive and spreads quickly into the myometrium
and the lymphatic system
. Thus even in presumed early stages, lymphadenectomy and omentectomy should be included in the surgical approach. If the tumor has spread surgery is cytoreductive followed by radiation therapy and/or chemotherapy.
In a study to determine if adjuvant therapy should be used in patients with stage I UPSC who had undergone surgery, no increased survival was seen when radiation therapy
was added versus observation, while the postsurgical treatment with chemotherapy may be beneficial but more data are needed.
A study of the usefulness of platinum
-based chemotherapy as an adjuvant after surgery of stage I patients showed that patients with stage 1A who had no residual disease in the hysterectomy specimen had no recurrence regardless if chemotherapy was used or not, however, patients with stage 1A disease with residual disease in the hysterectomy specimen had no recurrence with platinum-based therapy, but those who had no such chemotherapy showed recurrence in 43%. Similarly, patients with stage 1B disease with chemotherapy had no recurrence, while those without chemotherapy had a high degree (77%) of recurrence.
--the same one that is overexpressed in some breast cancer
s. The monoclonal antibody trastuzumab
(Herceptin) is currently being tested as a therapy for this subset of USCs.
The antibody trastuzumab
(Herceptin), which is used to treat breast cancers that overexpress the HER2/neu
protein, has been tried with some success in a phase II trial in women with UPSCs that overexpress HER2/neu.
Endometrial cancer
Endometrial cancer refers to several types of malignancies that arise from the endometrium, or lining, of the uterus. Endometrial cancers are the most common gynecologic cancers in the United States, with over 35,000 women diagnosed each year. The incidence is on a slow rise secondary to the...
that typically arises in postmenopausal women.
It is typically diagnosed on endometrial biopsy
Endometrial biopsy
The endometrial biopsy is a medical office procedure that is used to remove a sample of the lining of the uterus. The tissue subsequently undergoes a histologic evaluation which is supplied to the physician to aid in the diagnosis.-Indications:...
, prompted by post-menopausal bleeding.
Unlike the more common low-grade endometrioid endometrial adenocarcinoma, USC does not develop from endometrial hyperplasia
Endometrial hyperplasia
Endometrial hyperplasia is a condition of excessive proliferation of the cells of the endometrium, or inner lining of the uterus.Most cases of endometrial hyperplasia result from high levels of estrogens, combined with insufficient levels of the progesterone-like hormones which ordinarily...
and is not hormone-sensitive. It arises in the setting of endometrial atrophy
Atrophy
Atrophy is the partial or complete wasting away of a part of the body. Causes of atrophy include mutations , poor nourishment, poor circulation, loss of hormonal support, loss of nerve supply to the target organ, disuse or lack of exercise or disease intrinsic to the tissue itself...
and is classified as a type II endometrial cancer.
Diagnosis
The lesion is found in patients who present typically with abnormal or postmenopausal bleedingVaginal bleeding
Vaginal bleeding refers to bleeding in females that is either a physiologic response during the non-conceptional menstrual cycle or caused by hormonal or organic problems of the reproductive system. Vaginal bleeding may occur at any age, but always needs investigation when encountered in female...
. Such bleeding is followed by further evaluation leading to a tissue diagnosis, usually done by a dilatation and curettage (D&C). A work-up to follow would look for metastasis using imaging technology including sonography and MRI. The median age at diagnosis in a study of 138 women was 67 years, of these 54 had stage I, 20 stage II, 41 stage III, and 23 stage IV disease.
Histopathologically, uterine serous carcinomas is typically characterized by (1) nipple-shaped structures (papillae) with fibrovascular cores (2) marked nuclear atypia (irregularies in the nuclear membrane, enlarged nuclear size), (3) psammoma bodies and (4) cilia.
Staging
UPSC is staged like other forms of endometrial carcinoma at time of surgery using the FIGOInternational Federation of Gynecology and Obstetrics
The International Federation of Gynaecology and Obstetrics, usually just FIGO as the acronym of its French name Fédération Internationale de Gynécologie et d'Obstétrique, is a worldwide NGO organisation representing obstetricians and gynaecologists in over one hundred territories...
cancer staging
Cancer staging
The stage of a cancer is a description of the extent the cancer has spread. The stage often takes into account the size of a tumor, how deeply it has penetrated, whether it has invaded adjacent organs, how many lymph nodes it has metastasized to , and whether it has spread to distant organs...
system.
- Stage IA: tumor is limited to the endometrium
- Stage IB: invasion of less than half the myometriumMyometriumThe myometrium is the middle layer of the uterine wall, consisting mainly of uterine smooth muscle cells , but also of supporting stromal and vascular tissue...
- Stage IC: invasion of more than half the myometrium
- Stage IIA: endocervical glandular involvement only
- Stage IIB: cervical stromal invasion
- Stage IIIA: tumor invades serosa or adnexa, or malignant peritoneal cytology
- Stage IIIB: vaginal metastasis
- Stage IIIC: metastasis to pelvic or para-aortic lymph nodes
- Stage IVA: invasion of the bladder or bowel
- Stage IVB: distant metastasis, including intraabdominal or inguinal lymph nodes
Survival
In the older literature survival rates have been given as 35–50% for Stage I–II and 0–15% for Stage III and IV UPSC, More recently it was reported that forty-two percent of 138 patients were found disease-free at five years.In 2009, the journal of "Gynecologic Oncology" reported the following 5-year survival rates based upon stage of cancer:
- Stage I: 50% - 80%
- Stage II: 50%
- Stage III: 20%
- Stage IV: 5% - 10%
Treatment
The primary treatment is surgical. FIGO-cancer stagingCancer staging
The stage of a cancer is a description of the extent the cancer has spread. The stage often takes into account the size of a tumor, how deeply it has penetrated, whether it has invaded adjacent organs, how many lymph nodes it has metastasized to , and whether it has spread to distant organs...
is done at the time of surgery which consists of peritoneal cytology, total hysterectomy
Hysterectomy
A hysterectomy is the surgical removal of the uterus, usually performed by a gynecologist. Hysterectomy may be total or partial...
, bilateral salpingo-oophorectomy, pelvic/para-aortic lymphadenectomy
Lymphadenectomy
Lymphadenectomy consists of the surgical removal of one or more groups of lymph nodes. It is almost always performed as part of the surgical management of cancer....
, and omentectomy. The tumor is aggressive and spreads quickly into the myometrium
Myometrium
The myometrium is the middle layer of the uterine wall, consisting mainly of uterine smooth muscle cells , but also of supporting stromal and vascular tissue...
and the lymphatic system
Lymphatic system
The lymphoid system is the part of the immune system comprising a network of conduits called lymphatic vessels that carry a clear fluid called lymph unidirectionally toward the heart. Lymphoid tissue is found in many organs, particularly the lymph nodes, and in the lymphoid follicles associated...
. Thus even in presumed early stages, lymphadenectomy and omentectomy should be included in the surgical approach. If the tumor has spread surgery is cytoreductive followed by radiation therapy and/or chemotherapy.
In a study to determine if adjuvant therapy should be used in patients with stage I UPSC who had undergone surgery, no increased survival was seen when radiation therapy
Radiation therapy
Radiation therapy , radiation oncology, or radiotherapy , sometimes abbreviated to XRT or DXT, is the medical use of ionizing radiation, generally as part of cancer treatment to control malignant cells.Radiation therapy is commonly applied to the cancerous tumor because of its ability to control...
was added versus observation, while the postsurgical treatment with chemotherapy may be beneficial but more data are needed.
A study of the usefulness of platinum
Platinum
Platinum is a chemical element with the chemical symbol Pt and an atomic number of 78. Its name is derived from the Spanish term platina del Pinto, which is literally translated into "little silver of the Pinto River." It is a dense, malleable, ductile, precious, gray-white transition metal...
-based chemotherapy as an adjuvant after surgery of stage I patients showed that patients with stage 1A who had no residual disease in the hysterectomy specimen had no recurrence regardless if chemotherapy was used or not, however, patients with stage 1A disease with residual disease in the hysterectomy specimen had no recurrence with platinum-based therapy, but those who had no such chemotherapy showed recurrence in 43%. Similarly, patients with stage 1B disease with chemotherapy had no recurrence, while those without chemotherapy had a high degree (77%) of recurrence.
Trastuzumab
In a recent study, about 60% of USCs were found to overexpress the protein HER2/neuHER2/neu
HER-2 also known as proto-oncogene Neu, receptor tyrosine-protein kinase erbB-2, CD340 or p185 is an enzyme that in humans is encoded by the ERBB2 gene. Over expression of this gene is correlated with higher aggressiveness in breast cancers...
--the same one that is overexpressed in some breast cancer
Breast cancer
Breast cancer is cancer originating from breast tissue, most commonly from the inner lining of milk ducts or the lobules that supply the ducts with milk. Cancers originating from ducts are known as ductal carcinomas; those originating from lobules are known as lobular carcinomas...
s. The monoclonal antibody trastuzumab
Trastuzumab
Trastuzumab is a monoclonal antibody that interferes with the HER2/neu receptor.The HER receptors are proteins that are embedded in the cell membrane and communicate molecular signals from outside the cell to inside the cell, and turn genes on and off...
(Herceptin) is currently being tested as a therapy for this subset of USCs.
The antibody trastuzumab
Trastuzumab
Trastuzumab is a monoclonal antibody that interferes with the HER2/neu receptor.The HER receptors are proteins that are embedded in the cell membrane and communicate molecular signals from outside the cell to inside the cell, and turn genes on and off...
(Herceptin), which is used to treat breast cancers that overexpress the HER2/neu
HER2/neu
HER-2 also known as proto-oncogene Neu, receptor tyrosine-protein kinase erbB-2, CD340 or p185 is an enzyme that in humans is encoded by the ERBB2 gene. Over expression of this gene is correlated with higher aggressiveness in breast cancers...
protein, has been tried with some success in a phase II trial in women with UPSCs that overexpress HER2/neu.