BACKGROUND Herlyn-Werner-Wunderlich(HWW)syndrome is a rare Müllerian duct anomaly,characterized by a combination of urogenital abnormalities.The occurrence of primary cervico-vaginal carcinomas in patients with H...BACKGROUND Herlyn-Werner-Wunderlich(HWW)syndrome is a rare Müllerian duct anomaly,characterized by a combination of urogenital abnormalities.The occurrence of primary cervico-vaginal carcinomas in patients with HWW syndrome is excep-tionally rare,posing significant challenges for screening,early diagnosis,and effective management.CASE SUMMARY We report a rare case of primary clear cell carcinoma of the vagina complicated in a 40-year-old woman with HWW syndrome.The patient presented with irregular vaginal bleeding for 4 years.On gynecological examination,an oblique vaginal septum was suspected.Surgical resection of the vaginal septum revealed a com-municating fistula and a tumor on the left vagina and the left side of the septum,which was confirmed as clear cell carcinoma.One month later,she underwent a radical hysterectomy,vaginectomy,bilateral salpingo-oophorectomy,and pelvic lymph node dissection.Due to significant side effects,she completed only one course of chemotherapy.A year later,lung metastasis was detected and continued to grow.A thoracoscopic wedge resection of the right upper lobe was performed 4 years after the initial surgery.We also conducted a systemic review of the lite-rature on primary cervical or vaginal carcinoma in HWW syndrome to explore this rare entity.CONCLUSION Cervico-vaginal adenocarcinomas in patients with HWW syndrome are occult,and require early surgical intervention and regular imaging surveillance.展开更多
Humoral Hypercalcemia of Malignancy (HHM) has been reported in association with a number of malignancies. In gynecologic malignancies, ovarian Clear Cell Carcinoma (CCC) is one of the most commonhistologic subtypes, w...Humoral Hypercalcemia of Malignancy (HHM) has been reported in association with a number of malignancies. In gynecologic malignancies, ovarian Clear Cell Carcinoma (CCC) is one of the most commonhistologic subtypes, whereas HHM caused by endometrial CCC is very rare. We report a case of endometrial CCC with HHM, with a low serum intact PTH level, elevated serum PTH-related Peptide (PTH-rP), and immunohistochemically demonstrated PTH-rP in the neoplasm.展开更多
BACKGROUND Clear cell adenocarcinoma of the urethra is a rare type of aggressive cancer with a poor prognosis.Clear cell carcinoma of the urethra represents less than 0.02%of all malignancies in women.Adenocarcinomas ...BACKGROUND Clear cell adenocarcinoma of the urethra is a rare type of aggressive cancer with a poor prognosis.Clear cell carcinoma of the urethra represents less than 0.02%of all malignancies in women.Adenocarcinomas account for 10%of female urethral carcinomas,of which 40%are the clear cell variant.Determining the presence or absence of certain mutations through genetic testing may predict whether a patient with cancer may benefit from a particular chemotherapy regimen.CASE SUMMARY A 40-year-old woman presented with a 3-year history of slow urinary flow and a 3-mo history of urinary urgency and frequency as well as gross hematuria.An abdominal and pelvic computed tomography scan demonstrated enlarged lymph nodes in the abdomen and pelvis.A biopsy of a left inguinal lymph node microscopically confirmed a metastatic adenocarcinoma of the urethra.Specialized genetic testing determined personalized chemotherapy.She was treated successfully with a non-platinum-based chemotherapy consisting of paclitaxel and bevacizumab.Following 3 cycles of paclitaxel and bevacizumab,she attained significant clinical improvement,and response by FDG-Positron emission tomography(PET)imaging showed a definite improvement in size and metabolic activity.She achieved complete response after 6 cycles of therapy by PET scan.The patient concluded 11 cycles of paclitaxel and bevacizumab,and a subsequent PET scan confirmed progression of metastatic disease.The patient was then treated with two cycles of doxorubicin after which a PET scan revealed a mixed response to the treatment.CONCLUSION We report the first case of a patient with metastatic clear cell adenocarcinoma of the urethra who underwent personalized chemotherapy after testing for cancer gene alterations.Our unique case represents the safe and effective use of nonplatinum-based chemotherapy in clear cell adenocarcinoma of the urethra.展开更多
OBJECTIVE To assess the effect of 17 β-estradiol（E2） on cell proliferation, cell invasiveness and its regulation of MTA3, Snail and matrix metalloproteinase 2 （MMP-2） expression in the ovarian clear cell adenocar...OBJECTIVE To assess the effect of 17 β-estradiol（E2） on cell proliferation, cell invasiveness and its regulation of MTA3, Snail and matrix metalloproteinase 2 （MMP-2） expression in the ovarian clear cell adenocarcinoma cell line ES-2, and to further investigate the mechanism involved. METHODS We first investigated expression of ERα, ERβ, PR and E-cadherin of ES-2 cells by RT-PCR and Western blots. Before all experiments, the ES-2 cells were grown in medium depleted of steroid for more than 7 days. Following treatment with 10^-7,10^-8 and 10^-9 M E2, cell viability of the ES-2 cells was determined by the MTT method, and the cell cycle distribution and apoptosis were examined by flow cytometry （FCM）. Invasion and mobility assays were performed using modified Boyden chambers. MTA3, Snail and MMP-2 mRNA expression was measured by RT-PCR, and Snail, MMP-2 protein levels were determined by IHC. MMP-2 activity was assayed by zymography. RESULTS RT-PCR and Western Blots showed that theexpression of ERα and E-cadherin mRNA and protein in the ES-2 cells was negative, while ERβ and PR expression was positive. E2 at 10^-7,10^-8 or 10^-9M stimulated cell proliferation. A level of 10^-8M E2 reduced the proportion of G0-G1 phase cells and increased the proportion of cells in the S phase, but it had no effect on apoptosis. Invasiveness and mobility of the ES-2 cells was significantly increased by 10^-8M E2. Treatment with 10^-8M E2 led to reduced MTA3 mRNA expression, and elevated Snail and MMP-2 mRNA and protein levels. CONCLUSION E2 enhanced invasion by the ES-2 cells. The effects observed maybe mediated by down-regulation of MTA3 and up-reguation of Snail and MMP-2.展开更多
A 62-year-old woman with a history of curatively treated mucinous ovarian cancer, presented with dyspnea, anorexia and right-upper-quadrant pain at consultation with her general practitioner. A CT scan revealed severa...A 62-year-old woman with a history of curatively treated mucinous ovarian cancer, presented with dyspnea, anorexia and right-upper-quadrant pain at consultation with her general practitioner. A CT scan revealed several lymph node metastases in lungs and abdomen as well as a tumor in Morrison’s pouch and biopsy revealed renal cell carcinoma. Therefore, she was referred to Department of Urology. The multidisciplinary team could not immediately reject that there could be an exophytic tumor in the right kidney but discrepancy between histology and imaging, led to several biopsies including laparoscopic procedure. Re-examination of the primary ovarian cancer showed that one percent was classified as clear cell carcinoma. The final diagnose was metastatic clear cell ovarian carcinoma. The patient was terminal and suffered of cachexia and pleural effusion. The patient deceased four months after first consultation.展开更多
A 17-year-old female presented with rectal bleeding from an ulcerated sigmoid mass in 1994.Initial pathological evaluation revealed a rare clear cell neoplasm of the colon,possibly originating from kidneys,adrenals,lu...A 17-year-old female presented with rectal bleeding from an ulcerated sigmoid mass in 1994.Initial pathological evaluation revealed a rare clear cell neoplasm of the colon,possibly originating from kidneys,adrenals,lung or a gynecologic source as a metastatic lesion.Extensive imaging studies were negative,and over the next 15 years,she remained well with no recurrence.The original resected neoplasm was reviewed and reclassified as a perivascular epithelioid cell neoplasm (PEComa).Although the long-term natural history of PEComas requires definition,increased clinical and pathological awareness should lead to increased recognition of an apparently rare type of colonic neoplasm that likely occurs more often than is currently appreciated.展开更多
文摘BACKGROUND Herlyn-Werner-Wunderlich(HWW)syndrome is a rare Müllerian duct anomaly,characterized by a combination of urogenital abnormalities.The occurrence of primary cervico-vaginal carcinomas in patients with HWW syndrome is excep-tionally rare,posing significant challenges for screening,early diagnosis,and effective management.CASE SUMMARY We report a rare case of primary clear cell carcinoma of the vagina complicated in a 40-year-old woman with HWW syndrome.The patient presented with irregular vaginal bleeding for 4 years.On gynecological examination,an oblique vaginal septum was suspected.Surgical resection of the vaginal septum revealed a com-municating fistula and a tumor on the left vagina and the left side of the septum,which was confirmed as clear cell carcinoma.One month later,she underwent a radical hysterectomy,vaginectomy,bilateral salpingo-oophorectomy,and pelvic lymph node dissection.Due to significant side effects,she completed only one course of chemotherapy.A year later,lung metastasis was detected and continued to grow.A thoracoscopic wedge resection of the right upper lobe was performed 4 years after the initial surgery.We also conducted a systemic review of the lite-rature on primary cervical or vaginal carcinoma in HWW syndrome to explore this rare entity.CONCLUSION Cervico-vaginal adenocarcinomas in patients with HWW syndrome are occult,and require early surgical intervention and regular imaging surveillance.
文摘Humoral Hypercalcemia of Malignancy (HHM) has been reported in association with a number of malignancies. In gynecologic malignancies, ovarian Clear Cell Carcinoma (CCC) is one of the most commonhistologic subtypes, whereas HHM caused by endometrial CCC is very rare. We report a case of endometrial CCC with HHM, with a low serum intact PTH level, elevated serum PTH-related Peptide (PTH-rP), and immunohistochemically demonstrated PTH-rP in the neoplasm.
文摘BACKGROUND Clear cell adenocarcinoma of the urethra is a rare type of aggressive cancer with a poor prognosis.Clear cell carcinoma of the urethra represents less than 0.02%of all malignancies in women.Adenocarcinomas account for 10%of female urethral carcinomas,of which 40%are the clear cell variant.Determining the presence or absence of certain mutations through genetic testing may predict whether a patient with cancer may benefit from a particular chemotherapy regimen.CASE SUMMARY A 40-year-old woman presented with a 3-year history of slow urinary flow and a 3-mo history of urinary urgency and frequency as well as gross hematuria.An abdominal and pelvic computed tomography scan demonstrated enlarged lymph nodes in the abdomen and pelvis.A biopsy of a left inguinal lymph node microscopically confirmed a metastatic adenocarcinoma of the urethra.Specialized genetic testing determined personalized chemotherapy.She was treated successfully with a non-platinum-based chemotherapy consisting of paclitaxel and bevacizumab.Following 3 cycles of paclitaxel and bevacizumab,she attained significant clinical improvement,and response by FDG-Positron emission tomography(PET)imaging showed a definite improvement in size and metabolic activity.She achieved complete response after 6 cycles of therapy by PET scan.The patient concluded 11 cycles of paclitaxel and bevacizumab,and a subsequent PET scan confirmed progression of metastatic disease.The patient was then treated with two cycles of doxorubicin after which a PET scan revealed a mixed response to the treatment.CONCLUSION We report the first case of a patient with metastatic clear cell adenocarcinoma of the urethra who underwent personalized chemotherapy after testing for cancer gene alterations.Our unique case represents the safe and effective use of nonplatinum-based chemotherapy in clear cell adenocarcinoma of the urethra.
文摘OBJECTIVE To assess the effect of 17 β-estradiol（E2） on cell proliferation, cell invasiveness and its regulation of MTA3, Snail and matrix metalloproteinase 2 （MMP-2） expression in the ovarian clear cell adenocarcinoma cell line ES-2, and to further investigate the mechanism involved. METHODS We first investigated expression of ERα, ERβ, PR and E-cadherin of ES-2 cells by RT-PCR and Western blots. Before all experiments, the ES-2 cells were grown in medium depleted of steroid for more than 7 days. Following treatment with 10^-7,10^-8 and 10^-9 M E2, cell viability of the ES-2 cells was determined by the MTT method, and the cell cycle distribution and apoptosis were examined by flow cytometry （FCM）. Invasion and mobility assays were performed using modified Boyden chambers. MTA3, Snail and MMP-2 mRNA expression was measured by RT-PCR, and Snail, MMP-2 protein levels were determined by IHC. MMP-2 activity was assayed by zymography. RESULTS RT-PCR and Western Blots showed that theexpression of ERα and E-cadherin mRNA and protein in the ES-2 cells was negative, while ERβ and PR expression was positive. E2 at 10^-7,10^-8 or 10^-9M stimulated cell proliferation. A level of 10^-8M E2 reduced the proportion of G0-G1 phase cells and increased the proportion of cells in the S phase, but it had no effect on apoptosis. Invasiveness and mobility of the ES-2 cells was significantly increased by 10^-8M E2. Treatment with 10^-8M E2 led to reduced MTA3 mRNA expression, and elevated Snail and MMP-2 mRNA and protein levels. CONCLUSION E2 enhanced invasion by the ES-2 cells. The effects observed maybe mediated by down-regulation of MTA3 and up-reguation of Snail and MMP-2.
文摘A 62-year-old woman with a history of curatively treated mucinous ovarian cancer, presented with dyspnea, anorexia and right-upper-quadrant pain at consultation with her general practitioner. A CT scan revealed several lymph node metastases in lungs and abdomen as well as a tumor in Morrison’s pouch and biopsy revealed renal cell carcinoma. Therefore, she was referred to Department of Urology. The multidisciplinary team could not immediately reject that there could be an exophytic tumor in the right kidney but discrepancy between histology and imaging, led to several biopsies including laparoscopic procedure. Re-examination of the primary ovarian cancer showed that one percent was classified as clear cell carcinoma. The final diagnose was metastatic clear cell ovarian carcinoma. The patient was terminal and suffered of cachexia and pleural effusion. The patient deceased four months after first consultation.
文摘A 17-year-old female presented with rectal bleeding from an ulcerated sigmoid mass in 1994.Initial pathological evaluation revealed a rare clear cell neoplasm of the colon,possibly originating from kidneys,adrenals,lung or a gynecologic source as a metastatic lesion.Extensive imaging studies were negative,and over the next 15 years,she remained well with no recurrence.The original resected neoplasm was reviewed and reclassified as a perivascular epithelioid cell neoplasm (PEComa).Although the long-term natural history of PEComas requires definition,increased clinical and pathological awareness should lead to increased recognition of an apparently rare type of colonic neoplasm that likely occurs more often than is currently appreciated.
