Giant cell arteritis of the female genital tract.

Giant cell arteritis (GCA) of the female genital tract is rare with only 30 cases, including this case, documented in the literature. We present a patient who had total abdominal hysterectomy and bilateral salpingo-oophorectomy for an ovarian cyst and on histologic examination GCA involving the arteries of the myometrium, cervical stroma, ovaries and tubes was unexpectedly discovered. Upon questioning of the patient and further investigations it became clear that the patient did have symptoms and signs suggestive of systemic GCA including fatigue, low-grade fever, weight loss and elevated erythrocyte sedimentation rate (ESR). Treatment with oral corticosteroids resulted in rapid and complete recovery. It is concluded that an incidental finding of GCA in the genitalia should alert the clinician to the possibility of systemic GCA. If upon questioning of the patient and further investigations the existence of systemic GCA is confirmed, treatment with corticosteroids should be considered.

Primary malignant melanoma of the vagina: case report and review of literature.

Primary vaginal malignant melanoma is rare, with < 250 reported cases to date. It accounts for < 1% of all melanomas in women, < 10% of all female genital tract melanomas, and < 3% of all vaginal malignancies. Its clinical behavior is notoriously more aggressive than that of cutaneous and vulvar melanoma, with a 5-year survival rate ranging from 5% to 25%. Tumor size is the strongest predictor of survival, whereas tumor thickness is a weak predictor of survival. A case of F.I.G.O. stage I vaginal melanoma encircling and embracing the entire circumference of the middle third of the vagina is described. Tumor size and thickness were 6 cm and 9 mm, respectively. The patient was treated by wide local excision and pelvic radiotherapy, and to date, 11 months after surgery, she is alive and with no evidence of disease. It is concluded that wide local excision followed by pelvic radiotherapy is an appropriate treatment for melanoma confined to the vagina.

Peritoneal tuberculosis mimicking ovarian carcinoma with ascites and elevated serum CA-125: case report and review of literature.

Peritoneal tuberculosis is rare in the western world, although its incidence has been increasing in recent years. The presenting signs and symptoms, imaging examinations and CA-125 status in peritoneal tuberculosis may resemble that of ovarian carcinoma. Thus, the possibility of peritoneal tuberculosis should be considered in the differential diagnosis of ovarian carcinoma, especially in women immigrants from countries with a high prevalence of tuberculosis. A case of peritoneal tuberculosis mimicking ovarian carcinoma in a young woman immigrant from India is described. The patient presented with ascites, abdominopelvic masses and elevated serum CA-125 (1,081 U/ml). Laparoscopy confirmed the diagnosis of peritoneal tuberculosis and no malignancy, and thereby unnecessary extended surgery was avoided. Following treatment with the 4-drug anti-tuberculosis regimen, the patient recovered with disappearance of ascites and abdominopelvic masses, and return of CA-125 to normal range. It is concluded that laparoscopy, if feasible, seems to be a sufficient and safe method to provide diagnosis of peritoneal tuberculosis.

Primary breast carcinoma of the vulva: case report and review of literature.

The occurrence of ectopic breast tissue within the vulva is uncommon and the development of breast carcinoma within vulvar ectopic breast tissue is very rare. To date, only 12 cases of primary vulvar breast carcinoma have previously been reported in the English literature. This paper presents the 13th reported case of primary breast carcinoma of the vulva. The patient presented with a vulvar ulcerated lump and the diagnosis was based on a morphologic pattern consistent with breast carcinoma and the presence of estrogen and progesterone receptors. Primary surgery consisted of radical vulvectomy and bilateral groin dissection. The groin lymph nodes were involved bilaterally. Adjuvant therapy consisted of systemic chemotherapy (4 cycles of adriamycin and cyclophosphamide followed by 4 cycles of paclitaxel) and pelvic radiotherapy. Oral tamoxifen 20 mg/day was started for the next five years. It is concluded that the management of primary breast carcinoma of the vulva should be modeled after that for primary carcinoma of the orthotopic breast with primary surgery followed by systemic chemotherapy and pelvic radiotherapy. Chemotherapy should be similar to that employed for breast carcinoma. Tamoxifen should be prescribed for patients whose tumors contain estrogen receptors.

Psammomacarcinoma of the peritoneum.

Psammomacarcinoma is a rare histologic subtype of serous carcinoma originating in the ovary or peritoneum, characterized by massive psammoma body formation, invasiveness, and low-grade differentiation. Its clinical behavior appears similar to that of serous borderline tumors rather than that of typical invasive serous carcinomas. Peritoneal psammomacarcinoma is even rarer than its ovarian counterpart with only 10 cases have previously been documented in the literature. A case of F.I.G.O. stage IIIC peritoneal psammomacarcinoma is described. Optimal debulking surgery, including omentectomy, total abdominal hysterectomy, bilateral adnexectomy, and resection of segment of jejunum, was performed. The patient received adjuvant chemotherapy with taxol and cisplatin, and to date, 15 months after surgery, she is alive and with no evidence of disease. It is concluded that peritoneal psammomacarcinoma is a very rare tumor that behaves less aggressively than typical serous carcinoma. The mainstay of treatment is surgical debulking. The role of adjuvant chemotherapy has as yet not been established.

Lymphoma-like lesion of the uterine cervix.

Lymphoma-like lesion of the uterine cervix is a benign reactive lymphoid hyperplasia associated with chronic cervicitis that may pose a problem in differential diagnosis from malignant lymphoma. It is a rare entity and only about 20 cases have been reported in the world literature. Two cases of cervical lymphoma-like lesion are described. In both patients, the diagnosis was based on microscopic examination of biopsies taken from the uterine cervix. One patient subsequently had a cervical conization that confirmed the diagnosis of lymphoma-like lesion. Both patients are well, alive and with no evidence of malignant lymphoma. It is concluded that the diagnosis of lymphoma-like lesion is based on the well-established characteristic microscopic features of this lesion. Immunohistochemical staining is usually not helpful in distinguishing this lesion from malignant lymphoma. No specific treatment is necessary for this condition.

Three primary malignancies related to BRCA mutation successively occurring in a BRCA1 185delAG mutation carrier.

The 185delAG and 5382insC mutations in the BRCA1 gene and the 6174delT mutation in the BRCA2 gene (the Ashkenazi mutations) have been found to be significantly more common among Jews of eastern European ancestry (1 in 40, 2.5%) in comparison to the general population (1 in 800 to 1 in 300, 0.12-0.33%). Carriers of these mutations, especially the BRCA1 185delAG mutation, have a significantly increased lifetime risk of breast and ovarian carcinoma and other carcinomas as compared to non-carriers. A case of three primary malignancies related to the BRCA1 185delAG mutation successively occurring in a carrier of this mutation, is described. The patient successively developed breast carcinoma, ovarian micropapillary serous carcinoma and peritoneal papillary serous carcinoma. Immunohistochemical staining results have indicated that these tumors are three separate primary malignancies. This case illustrates that ovarian serous borderline tumors (including micropapillary serous carcinoma) and peritoneal papillary serous carcinomas should be considered, like breast and ovarian carcinomas, tumors expressed in BRCA mutation carriers.

Micropapillary serous carcinoma of the ovary: case report and review of literature.

The term "micropapillary serous carcinoma" (MPSC) has recently been introduced to define a subset of ovarian serous borderline tumors morphologically characterized by a micropapillary pattern and clinically associated with a more aggressive behavior than that of the typical ovarian serous borderline tumors. Ovarian MPSC's are associated with extra-ovarian invasive peritoneal implants and invasive recurrences much more frequently than typical ovarian serous borderline tumors. The case of a women, who at age 28 had bilateral ovarian cystectomy and four months later total abdominal hysterectomy and bilateral salpingo-oophorectomy for bilateral ovarian MPSC, is reported. She was free of disease for the next 15 years and then presented with a central pelvic mass. At laparotomy, a recurrence in the form of a solitary invasive peritoneal implant was discovered and completely resected. No postoperative adjuvant therapy was given. To date, 16 years after initial diagnosis of MPSC, and one year after detection of recurrence, the patient is alive, well and without disease. Literature data and this case report support the view that MPSC's should be classified separately from both typical serous borderline tumors and invasive carcinomas of the ovary.

Mullerian adenosarcoma of the uterus: case report and review of literature.

Mullerian adenosarcoma--a variant of mullerian mixed mesodermal tumor of the uterus--is typically composed of benign but sometimes mildly atypical glandular epithelial elements admixed with malignant sarcomatous stroma. This rare tumor, which accounts for only about 8% of all uterine sarcomas, usually originates in the endometrium and grows as a polypoid mass within the endometrial cavity. The most prevailing presenting symptom is abnormal vaginal bleeding and the most common finding is a polypoid mass protruding through a dilated cervical canal. The case of a woman, who at age 62 presented with symptoms and signs of acute pelvic inflammatory disease and on vaginal examination an infected mullerian adenosarcoma protruding through a dilated cervical canal was discovered, is reported. Treatment consisted of extensive antibiotic treatment and surgery comprised of total abdominal hysterectomy and bilateral salpingo-oophorectomy followed by postoperative adjuvant pelvic radiotherapy. One year later, the patient is alive with no evidence of disease.

Mucosal epithelial proliferation of the fallopian tube: prevalence, clinical associations, and optimal strategy for histopathologic assessment.

The prevalence and clinical significance of mucosal epithelial proliferation or hyperplasia of the fallopian tube are controversial in the few studies reported. Some authors have retrospectively examined "routine" sections (one or two submitted from each tube), whereas others have prospectively blocked the entire tubes. In the current study, we prospectively studied a total of 168 tubes from 98 women who had various indications for salpingectomy and compared the diagnosis in an initial single section (to simulate the usual practice) with that in the remainder of the entirely sectioned and submitted tube (mean total number of sections, 9.0). Some degree of mucosal epithelial proliferation was found in 83% of all tubes examined, with no difference between the tubes removed for routine tubal ligation and those in women who had benign ovarian lesions, malignant gynecologic tumors, uterine leiomyomata, or benign tubal lesions (salpingitis or ectopic pregnancy). Mucosal epithelial proliferation graded as more than mild, however, was seen in only 4.5% of the otherwise normal ligated tubes versus 35 to 46% of tubes associated with the other lesions. When the initial sections were compared with the subsequent ones, the diagnosis was identical in 96 tubes (57%). In the other 72 tubes (43%), the difference in diagnosis was never greater than one grade (no, mild, moderate, severe mucosal epithelial proliferation), with the diagnosis more often upgraded (50 tubes) than downgraded (22 tubes) in the additional sections. It is concluded that there is no reason to submit an entire tube for histologic examination to detect clinically significant lesions, and the usual practice of submission of one or two sections is clinically appropriate.

Glassy cell carcinoma of the uterine cervix.

BACKGROUND AND OBJECTIVES: Glassy cell carcinoma (GCC) of the uterine cervix is a rare and highly malignant tumor, accounting for only 1%-2% of all cervical carcinomas. The purpose of this study was to investigate the clinical findings, treatment, and outcome of patients with cervical GCC in the south of Israel. METHODS: Data from the files of 5 patients with cervical GCC who were managed at the Soroka Medical Center, Beer-Sheva, Israel, between January 1961 and June 1999 were evaluated. RESULTS: Age at diagnosis ranged from 32 to 84 years, with 1 patient pregnant at the time of diagnosis. Vaginal bleeding was the prevailing presenting symptom. The cervical lesion was exophytic in 4 patients and endophytic ("barrel-shaped") in 1 patient. Mean tumor size was 3.9 cm. Three patients with stage IB(1) disease had radical hysterectomy and bilateral pelvic lymph node dissection followed by either external pelvic radiotherapy or brachytherapy or both. All 3 patients were alive without disease 4, 12, and 18 months after initial diagnosis, respectively. One patient with stage IIIB disease had external pelvic radiotherapy alone and died of disease 12 months after initial diagnosis. One patient with stage IVB disease refused treatment and died of disease 3 months after initial diagnosis. CONCLUSIONS: Cervical GCC is a rare variant of cervical cancer with distinct histologic features and an alleged aggressive clinical behavior. For early-stage disease, the treatment of choice seems to be radical surgery followed by chemoradiotherapy.

Sigmoid colon carcinoma metastatic to the myometrium.

Metastases to the uterus from extragenital cancers are significantly rarer than metastases to the ovaries. Of the approximately 200 cases of metastases to the uterus from extragenital cancers that have been reported in the literature, more than half are cases of metastases from breast carcinoma and only 16 are cases of metastases from colorectal carcinoma. A case of isolated metastases restricted to the myometrium of the right uterine comu from sigmoid colon carcinoma, without involvement of the ovaries, is described. The patient who six months previously had surgery for sigmoid colon carcinoma presented with right lower abdominal pain and a palpable mass in the region of the right uterine cornu. The diagnosis of isolated metastases restricted to the myometrium of the right uterine cornu was confirmed by total abdominal hysterectomy and bilateral salpingo-oophorectomy. This case illustrates that a growing uterine mass in a patient with a history of primary extragenital cancer, regardless of whether abnormal uterine bleeding is present or absent, should alert the physician to consider the possibility of uterine metastases.

Abnormal uterine bleeding as a presenting sign of metastases to the uterine corpus, cervix and vagina in a breast cancer patient on tamoxifen therapy.

Metastases to the female genital tract from extragenital cancers are uncommon. The ovaries are most often affected with the breast and gastrointestinal tract being the most common sites of the primary malignancy. Metastases to the uterus from extragenital cancers are significantly rarer than metastases to the ovaries and in the majority of cases the ovaries are also involved. A case of metastases restricted to the uterine corpus, cervix and vagina from breast carcinoma, without involvement of the ovaries, is described. The patient who had been on tamoxifen therapy presented with postmenopausal bleeding. The diagnosis of uterine metastases was established during endometrial ablation and confirmed by total abdominal hysterectomy and bilateral salpingo-oophorectomy. This case illustrates that abnormal uterine bleeding in a breast cancer patient, regardless of whether she is receiving or not receiving tamoxifen, should always alert the physician to consider the possibility of uterine metastases from breast carcinoma.

Primary hepatoid carcinoma of ovary in pregnancy.

We report a rare case of a 35-year-old woman with primary hepatoid carcinoma of the ovary during pregnancy associated with abnormal levels of maternal serum alpha-fetoprotein. This tumor should be considered in the differential diagnosis of pregnant women with very high levels of maternal serum alpha-fetoprotein whenever other maternal or fetal sources are excluded.

Peritoneal papillary serous carcinoma: study of 15 cases and comparison with stage III-IV ovarian papillary serous carcinoma.

BACKGROUND AND OBJECTIVES: Peritoneal papillary serous carcinoma (PPSC) is histologically and clinically similar to stage III-IV ovarian papillary serous carcinoma (OPSC). The purpose of this study was to investigate the clinical findings, treatment, and outcome of PPSC patients compared with stage III-IV OPSC patients. METHODS: Data from the files of 15 PPSC patients and 52 stage III-IV OPSC patients who were managed at the Soroka Medical Center between January 1991 and December 1997 were evaluated. RESULTS: With regard to patients' characteristics, presenting signs and symptoms, type and extent of surgery, tumor response to first-line chemotherapy, recurrence-free interval, recurrence site, tumor response to second-line chemotherapy, and serum CA-125 levels, no significant differences were observed between the PPSC patients and the stage III-IV OPSC controls. The prevailing presenting symptoms were abdominal mass and ascites. The mainstay of treatment was debulking surgery followed by adjuvant platinum-containing chemotherapy. The objective response rate to first-line chemotherapy was 80%. The actuarial 5-year survival rate for the PPSC patients and stage III-IV OPSC patients was 52.0% and 20.5%, respectively (0.05 < P < 0.1). CONCLUSIONS: The clinical and surgical characteristics of patients with PPSC are similar to those of patients with stage III-IV OPSC. When treatment strategies for stage III-IV OPSC are applied to PPSC, the survival of PPSC patients may be similar or even better than that of stage III-IV OPSC patients.

Tumor necrosis factor-alpha and interleukin-6 are differently expressed by fresh human cancerous ovarian tissue and primary cell lines.

The expression levels of tumor necrosis factor-alpha (TNF-alpha) and interleukin-6 (IL-6) were evaluated in normal and cancerous ovarian tissue and primary cell lines (PCL). Seven normal and 10 cancerous, formalin-fixed ovarian samples were examined for TNF-alpha and IL-6 expression by immunohistochemical staining using polyclonal rabbit anti-human TNF-alpha and IL-6 antibodies. Fresh normal and cancerous specimens were also examined for TNF-alpha and IL-6 secretion by bioassay and immunoassay prior to and following in vitro stimulation with lipopolysaccharide (LPS). The levels of IL-6 and TNF-alpha were higher in cancerous tissues than in normal specimens. In vitro stimulation of normal and cancerous ovarian tissues and PCL revealed their capacity to secrete IL-6. Cancerous tissue and PCL secreted higher levels than normal tissue and PCL. Stimulation of both groups with LPS increased their capacity to secrete IL-6. Optimal secretion of IL-6 by the cancerous tissue was observed after 72 hours with or without LPS (10 microg/ml). Normal tissue secreted maximal levels of IL-6 after 96 hours with or without LPS. PCL from normal ovarian tissue secreted IL-6 constitutively and optimal expression was detected after 96 hours. Carcinoma PCL from cancerous ovarian tissue demonstrated optimal secretion of IL-6 after 24 hours. Stimulation of both types of cells with LPS, IL-1 or TNF-alpha increased their capacity to secrete IL-6. TNF-alpha activity was detected in vitro only in supernatants of ovarian cancerous tissue and only after LPS stimulation; optimal levels were detected after 48 hours and 1 microg/ml LPS. Our results indicate that IL-6 and TNF-alpha are expressed in cancerous ovarian tissue at a higher level than in normal ovarian tissues. Carcinoma cells of ovarian tissues are the main cellular source of these cytokines. The conditions controlling the secretion of these cytokines, under in vitro conditions, are different in cancerous and normal ovarian tissues.

Primary sarcoma of the ovary: report of five cases and review of the literature.

Primary ovarian sarcomas are rare and usually behave very aggressively. Over a ten-year period (1987-1996) five cases of primary ovarian sarcoma were managed at the Soroka Medical Center, Beer-Sheva, Israel. Four patients had malignant mullerian-mixed mesodermal tumor (MMMMT): two had Stage IIIC tumor with chondrosarcoma being the predominant sarcomatous element, one had Stage IIIC tumor with high-grade endometrioid stromal sarcoma (ESS) being the predominant sarcomatous element and one had Stage IC tumor with rhabdomyosarcoma being the predominant sarcomatous element. One patient had Stage IA leiomyosarcoma (LMS). All four patients with MMMMT received postoperative adjuvant chemotherapy, whereas the patient with LMS did not. The four patients with MMMMT died of their disease 10, 10, 13 and 25 months, respectively, after initial surgery. The patient with LMS died of intercurrent disease 21 months after initial surgery. It is concluded that most patients with primary ovarian sarcoma present with extraovarian disease and the prognosis is poor. The mainstay of treatment is debulking surgery consisting of total abdominal hysterectomy, bilateral salpingo-oophorectomy and extirpation of tumor masses. The benefit of postoperative adjuvant chemotherapy and/or radiotherapy is still a subject of debate and has yet not been established.

Uterine papillary serous carcinoma: study of 19 cases.

OBJECTIVE: Uterine papillary serous carcinoma (UPSC) is an uncommon highly malignant variant of endometrial carcinoma that histologically and clinically resembles ovarian papillary serous carcinoma. The purpose of this study was to present the conjoined experience of two regional hospitals in the south of Israel (Soroka Medical Center, Beer-Sheva and Kaplan Hospital, Rehovot) of handling this tumour. STUDY DESIGN: Data from the files of 19 patients with UPSC who were managed at these hospitals between July 1991 and June 1997 were evaluated. RESULTS: The three-year survival rate was 57.3% overall; 83.3% for Stage I and 21.2% for Stages II, III, and IV combined (P<0.02). Eighteen patients had primary surgery which included total abdominal hysterectomy and bilateral salpingo-oophorectomy and 15 (83.3%) of them received postoperative adjuvant therapy which included radiotherapy and/or systemic chemotherapy. CONCLUSIONS: The prognosis of patients with UPSC is worse than that of patients with other forms of endometrial carcinoma. Primary surgery comprised of total abdominal hysterectomy, bilateral salpingo-oophorectomy and staging is the mainstay of treatment. The type of postoperative treatment is not consistent. By and large, adjuvant pelvic radiotherapy is usually given in early-stage disease and adjuvant systemic chemotherapy is usually prescribed in advanced-stage disease.

Clinical-pathological study of uterine leiomyomas with high mitotic activity.

OBJECTIVE: To evaluate clinical-pathological aspects of mitotically active leiomyomas. STUDY DESIGN: Twenty patients with smooth muscle tumors of the uterus, with 5-9 mitotic figures per 10 high power fields and without atypia or necrosis were studied. The clinical and pathological characteristics of these tumors were reviewed and analyzed. RESULTS: Patients' ages ranged from 33 to 63 years (mean 42.5 years). The size of the tumors ranged from 1.5 to 9.5 cm (mean 5.4 cm). On gross examination all tumors appeared as typical leiomyomas. Treatment included hysterectomy in 16 patients and myomectomy in four. Follow-up periods ranged from 1.5 to 11 years (mean 6.8 years). None of the patients developed a recurrent tumor. CONCLUSION: The benign clinical behavior of such tumors supports their current designation as mitotically active leiomyomas, thus deleting the previous misnomer 'smooth muscle tumors of uncertain malignant potential'. Myomectomy is an appropriate treatment, particularly in young patients interested in reproduction.