Desmoplastic small round cell tumor: report of a case presenting as a primary ovarian neoplasm

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is an intra-abdominal malignancy that typically has extensive peritoneal spread at the time of diagnosis. We report a case of DSRCT with involvement of the ovary and omentum as well as an elevated CA-125 level at presentation. CASE: A 23-year-old female presented to another institution with a pelvic mass and a CA-125 level of 140 U/ml. During tumor reductive surgery the right ovary, omentum, and liver were found to be involved. Initial histologic examination favored an undifferentiated small cell carcinoma of the ovary. The patient received two cycles of Taxol and cisplatin chemotherapy and was referred to the University of Texas M. D. Anderson Cancer Center. Upon review of the pathology material at the time of the referral, a diagnosis of DSRCT was made. Despite two additional cycles of chemotherapy, the tumor progressed, and the patient returned home. CONCLUSION: DSRCT may mimic an ovarian primary tumor by presenting with involvement of the ovary and an elevated CA-125 level, and should be included in the differential diagnosis of ovarian neoplasms in young patients.     

Primary ovarian undifferentiated non-small cell carcinoma, neuroendocrine type

BACKGROUND: Non-small cell neuroendocrine carcinoma (NSCNEC) of the ovary is a rare aggressive neoplasm, characteristically arising in association with a surface epithelial tumor. CASE: A 27-year-old woman with no significant past medical history presented with a 17-cm left ovarian tumor. She underwent an exploratory laparotomy with resection of the pelvic mass. A diagnosis of stage IA non-small cell neuroendocrine carcinoma was rendered. She was subsequently treated with Taxol and Carboplatinum. CONCLUSION: This is a report of a rare primary ovarian non-small cell neuroendocrine carcinoma. In contrast to prior reports, this neoplasm was not associated with a surface epithelial tumor.     

Primary extrarenal rhabdoid tumor of the ovary. A case report

BACKGROUND: Malignant rhabdoid tumors are rare, aggressive neoplasms that consist of both renal and extrarenal subtypes. Although extrarenal rhabdoid tumors have been documented at multiple extrarenal sites, to our knowledge no primary ovarian cases have been reported. CASE: An 18-year-old, Caucasian woman was diagnosed with a pure primary extrarenal rhabdoid tumor of the ovary following diagnostic laparoscopy for pelvic pain. The tumor exhibited rapid growth, failed to respond to chemotherapy and led rapidly to death. CONCLUSION: Although no other reports on primary ovarian extrarenal rhabdoid tumor have been published, the aggressive behavior of the tumor in this patient was similar to that seen in patients with metastatic disease.     

Struma ovarii showing clinical characteristics of ovarian malignancy

Struma ovarii is a rare form of ovarian neoplasm, composed entirely or predominantly of thyroid tissue and generally a benign germ cell tumor of the ovary. Ascites have been reported in approximately 15-20% of all cases. However, the combination of struma ovarii and elevated CA125 has rarely been reported. We experienced a rare case of a postmenopausal woman with benign struma ovarii associated with massive ascites, a complex pelvic mass. There was marked elevation of her CA125 level. The clinical impression was ovarian malignancy. Surgical excision of the ovarian mass induced immediate resolution of the ascites and a normalization of the serum CA125 level. No recurrence of the ascites or of the tumor has been observed during the 10-month follow-up. Struma ovarii can mimic ovarian malignancy clinically, particularly if complex and associated with ascites and an elevated CA125 level.     

Ovarian neuroendocrine carcinoma, non-small cell type, associated with serous carcinoma

BACKGROUND: Neuroendocrine carcinoma of the non-small cell type of the ovary is a rare aggressive tumor, interestingly associated with either a surface epithelial tumor or teratoma. CASE: A 71-year-old woman presented with a pelvic mass and underwent a total abdominal hysterectomy with a bilateral salpingo-oophorectomy. Pathology examination showed a 6.5 cm in greatest dimension ovarian tumor composed of neuroendocrine carcinoma of the non-small cell type and serous carcinoma. Immunohistochemical studies including keratin 7, WT-1, and neuroendocrine markers demonstrated differences in the two components. Microsatellite instability (MSI) analysis using five polymorphic markers also showed a different pattern in the two components. CONCLUSION: This is the first report of an ovarian neuroendocrine carcinoma, non-small cell type, associated with a serous carcinoma. Immunohistochemistry and MSI are very helpful in making a definite diagnosis.     

Endometrial metastasis of a primitive neuroendocrine ovarian carcinoma: management and treatment of a case

BACKGROUND: Neuroendocrine tumours are a heterogeneous group of separate clinico-pathological entities which have a common characteristic, i.e., expression of potential endocrine differentiation. In the ovary, the term "neuroendocrine" relates mainly to widely known carcinoids, but it may also be applied to rare neuroendocrine carcinomas as non-small cell type and small cell carcinomas of pulmonary type. In the literature only 11 cases of primary ovarian non-small cell neurendocrine carcinomas have been described and ten of these were associated with a surface epithelial ovarian tumour. Small cell neuroendocrine carcinoma of the ovary is a rare malignant tumour of the ovary. Advanced small cell carcinoma of the ovary is a very aggressive tumour with an overall poor prognosis and unfavourable outcome. CASE REPORT: The case reported is unique in the literature because the authors describe a rare case of endometrial metastasis of a primary ovarian non-small cell neurendocrine carcinoma without any surface epithelial ovarian tumour association. The tumour invaded up to less than half of the myometrium. The first symptoms were related to endometrial metastasis as metrorrhagia and pelvic pain while the asymptomatic presence of primary ovarian carcinoma was not acknowledged with physical examination, routine biochemistry, tumour markers, blood count and traditional transvaginal greyscale ultrasound. CONCLUSION: Magnetic resonance and three-dimensional (3D) ultrasonography with power Doppler are a great help in the diagnosis of ovarian localisation but only immunohistochemistry on histological material can provide a correct diagnosis. Immunohistochemistry expression of Ki67 is a useful marker of malignancy. Due to the rarity of this neoplasm, a general consensus for optimal treatment has yet to emerge. The reported biological aggressiveness of these tumours prompts combined treatment with radical surgery and adjuvant polychemotherapy.     

Bilateral ovarian fibromatosis presenting with ascites and hirsutism

BACKGROUND: Ovarian fibromatosis is a very rare nonneoplastic disease. Due to the rarity and atypical clinical presentations, they may give rise to a misdiagnosis of malignancy and unnecessary extensive surgical interventions. Literature lacks definitive data about this rare disease and its preoperative evaluations. MRI together with the intraoperative frozen section may help us to define the benign nature of the disease. In this report, we aimed to review the literature and give a highlight to the gynecologic oncologists about this rare disease. CASE HISTORY: A 19-year-old female patient admitted to our hospital with the complaints of menstrual irregularity, hirsutism, and increased abdominal girth. Physical examination revealed bilateral ovarian mass, hirsutism, and ascites. Serum CA-125 levels were slightly elevated. Preoperative MRI study showed bilateral hypointense lobulated ovarian masses. With the initial diagnosis of ovarian tumor, we performed explorative laparotomy and excised both masses. Final pathology was reported as bilateral ovarian fibromatosis. CONCLUSION: Ovarian fibromatosis commonly presents with ascites and solid pelvic mass and can be misdiagnosed as a malignant ovarian tumor. In young patients, clinicians should consider ovarian fibromatosis in differential diagnosis of pelvic mass. Preoperative MRI findings and intraoperative frozen examination may be used to avoid unnecessary aggressive surgical management.     

Renin-producing serous cystoadenocarcinoma of the ovary: a case report

BACKGROUND: Only a few renin-producing ovarian tumors have been reported, and most such ovarian tumors have been sex cord/stromal tumors. Renin-producing ovarian epithelial tumors are quite rare. CASE: A 46-year-old woman presented with hypertension and hypokalemia. Examinations of the patient revealed elevated plasma renin activity, hyperaldosteronism and a pelvic mass. Subsequently, a right ovarian tumor mass was resected. Microscopic observation of the tumor revealed a well-differentiated serous cystadenocarcinoma. Immediately after surgery, blood pressure, serum potassium, plasma renin activity and plasma aldosterone levels returned to normal ranges. RT-PCR analysis and immunohistochemical staining of this tumor indicated that it was producing renin. CONCLUSION: This is the first report of a renin-producing ovarian serous cystadenocarcinoma.     

Metastatic Hurthle cell carcinoma in the abdomen masquerading as a primary ovarian tumor: a case report

Hurthle cell carcinoma, a variant of follicular carcinoma of the thyroid, has been regarded as an aggressive type of differentiated thyroid cancer. It is diagnosed histologically and regarded as a carcinoma by the presence of vascular invasion or capsular invasion. In this case report, a patient with a history of thyroid Hurthle cell carcinoma presented with what seemingly appeared to be a pelvic mass of gynecological origin, with a raised risk-of-malignancy index of 567. She underwent a laparotomy which revealed the presence of multiple masses in the form of nodules and lumps attached to her small bowel, sigmoid colon, omentum, and infracolic region, without the involvement of her pelvic organs or para-aortic lymph nodes. At frozen section, the masses were reported to be high-grade metastatic tumor, possibly a melanoma, unlikely to be a female genital tract tumor. These masses were resected along with a 15-cm section of small bowel and analyzed. They were diagnosed to be secondary lesions of Hurthle cell carcinoma of the thyroid. Hurthle cell carcinoma of the thyroid has not been known to be associated with the elevation of CA125 nor has it been known to metastasize to the small bowel and sigmoid colon.     

A patient presenting with a pelvic mass, elevated CA-125, and fever

BACKGROUND: Tuberculous peritonitis is a rare event which can mimic advanced stage ovarian cancer. A pelvic mass and an elevated CA-125 is suggestive of an ovarian malignancy; however, benign conditions may be discovered, especially in the premenopausal patient. CASE: A patient with a pelvic mass, ascites, and an elevated CA-125 underwent an exploratory laparotomy for presumed ovarian cancer. Final pathology revealed pelvic tuberculosis without any pulmonary involvement. Acid-fast bacilli were confirmed with polymerase chain reaction in the surgical specimen. DISCUSSION: Pelvic tuberculosis is an uncommon gynecologic condition that presents with ascites, a pelvic mass, and fever. An elevated CA-125 is not specific for ovarian malignancy.     

Juvenile granulosa cell tumor of the ovary associated with hypercalcemia

Introduction Juvenile granulosa cell tumors (JGCTs) are rare ovarian neoplasms that typically occur in children and young women. While most JGCTs are diagnosed in Stage IA and can be cured by unilateral oophorectomy alone, the remainders are diagnosed in more advanced stages and may exhibit aggressive behavior despite surgery and chemotherapy. Paraneoplastic hypercalcemia occurs in 5% of ovarian malignancies with small cell and clear cell carcinoma being the commonest ovarian tumors associated with hypercalcemia. Only one case of ovarian JGCT associated with hypercalcemia has previously been documented in the literature. Case report A 25-year-old married childless woman presented with a unilateral ovarian mass associated with profound hypercalcemia and suppressed serum parathyroid hormone-intact (PTH-i) level. After rectifying the hypercalcemia with saline hydration, furosemide, and anti-hypercalcemic drugs, the patient underwent unilateral salpingo-oophorectomy that demonstrated Stage IC ovarian JGCT. The patient received adjuvant chemotherapy with three courses of BEP (bleomycin, etoposide and cisplatin) and to date, ten months after surgery, she is disease-free, eucalcemic, and menstruating normally. Conclusion This is the second case reported in the literature of ovarian JGCT associated with hypercalcemia. Since serum PTH-i level was suppressed and the hypercalcemia did not recur after resection of the tumor, it is concluded that the hypercalcemia was caused by parathyroid hormone-related protein (PTH-rP) produced by the tumor. It seems that unilateral salpingo-oophorectomy followed by BEP chemotherapy is an appropriate treatment for unilateral Stage IC ovarian JGCT in women desiring to preserve ovarian function and childbearing capacity.     

Transitional cell ovarian carcinoma in a BRCA1 mutation carrier

BACKGROUND:BRCA1 mutation carriers are at high risk of developing epithelial ovarian cancer, but the transitional cell variant has not been previously reported in these patients.CASE:A nulligravid, perimenopausal woman underwent exploratory laparotomy for a pelvic mass, ascites, and omental caking. Intraoperatively, frozen section of a tumor implant revealed high-grade epithelial ovarian carcinoma. Optimal surgical cytoreduction was performed. The final surgical pathology confirmed International Federation of Gynecology and Obstetrics stage IIIC transitional cell ovarian carcinoma. Her family history was significant for a sister with premenopausal breast cancer and a paternal aunt with ovarian cancer. The patient was counseled and elected to undergo genetic testing. Comprehensive gene sequence analysis detected the germline BRCA1 5382insC mutation.CONCLUSION:Transitional cell ovarian carcinoma is a rare histologic variant of epithelial ovarian cancer that may occur in BRCA1 mutation carriers.     

Yolk sac tumor of the ovary associated with endometrioid carcinoma with metastasis to the vagina: a case report

BACKGROUND: Mixed yolk sac tumors of the ovary are biologically aggressive even in early stage disease. CASE: A 41-year-old woman presented with a large pelvic mass and anterior vaginal wall tumor. At surgery vaginal biopsies were performed followed by an exploratory laparotomy with resection of the mass. Pathology of the ovary revealed a primary yolk sac tumor associated with poorly differentiated endometrioid and undifferentiated carcinoma with vaginal metastasis only. She was initiated on bleomycin, etoposide, and cisplatin, with three additional cycles of etoposide and cisplatin. Initially the patient experienced a complete response, however her disease recurred and she currently is dead of her disease. CONCLUSION: To our knowledge this is the first case of a mixed ovarian germ cell tumor with vaginal metastasis.     

Transvaginal color Doppler sonography and CA-125 elevation in a patient with ovarian thecoma and ascites.

A postmenopausal patient presented with a pelvic mass and ascites. The CA-125 level was 329 U/ml, and transvaginal color Doppler sonography of the tumor vasculature suggested malignancy. At laparotomy, a luteinized thecoma and cytologically benign ascites were found. On rare occasions ovarian thecoma may be associated with ascites without hydrothorax. Preoperative evaluation of the patient with ascites and a pelvic mass may suggest malignancy, but histologic confirmation is necessary to exclude this rare association.     

Mullerian adenosarcoma with sarcomatous overgrowth of the cervix presenting as cervical polyp: a case report and review of the literature

An aggressive variant of adenosarcoma, mullerian adenosarcoma with sarcomatous overgrowth (MASO) in the cervix is extremely rare. This variant contains obvious, high-grade sarcoma in addition to a low-grade form. In this report, we describe a case of MASO of the uterine cervix and review the clinical and pathological features of these tumors. The patient was a 37-year-old woman with a cervical polypoid mass, which was morphologically considered as a benign endocervical polyp. Microscopically, polypoid cervical mass showed diffuse and dense malignant spindle cell proliferation around the benign endocervical glands and also an area of markedly anaplastic and pleomorphic spindle cell proliferation, so called, sarcomatous overgrowth. Total abdominal hysterectomy and bilateral salpingo-oophorectomy with pelvic lymph node dissection were performed. The patient has been followed-up and neither chemotherapy nor other adjuvant therapies have been administered. At present, she has been clinically free of disease for 9 months since she received surgery. It is extremely rare that MASO of the uterine cervix is presented in premenopausal woman. Gynecologists and pathologists should be aware of the difficulties associated with a delay in the diagnosis of MASO when the tumor is present as a benign looking cervical polyp.     

Malignant transformation of an ovarian mature cystic teratoma presenting as a rectal mass

BACKGROUND: Squamous cell carcinoma arising from malignant degeneration of a mature cystic teratoma is rare with a reported incidence of approximately 1-3%. The most common presenting symptoms are lower abdominal pain and increasing abdominal girth of several months' duration. Approximately 50% of the patients present with FIGO stage I while 35-38% present with stage III diseases. CASE: The case described herein represents an unusual presentation and initial diagnostic dilemma of locally aggressive squamous cell carcinoma arising in an ovarian dermoid cyst, with invasion into the distal rectum and anal canal causing rectal bleeding similar to the presentation of anal squamous cell carcinoma. Despite aggressive surgical management with posterior exenteration and optimal tumor debulking followed by 5040-cGy pelvic radiation utilizing 25-MV photons, the patient developed pelvic recurrence at the vaginal cuff 6 weeks after completion of her adjuvant radiotherapy. She subsequently failed cis-platinum single-agent chemotherapy and died 9 months after her initial surgery and diagnosis. CONCLUSION: Squamous cell carcinoma in the anal canal, diagnosed by colonoscopy or proctoscopy, could be an unusual presentation of that arising from malignant degeneration of an ovarian dermoid cyst. This tumor may behave in a locally aggressive manner and be resistant to pelvic radiation or single-agent chemotherapy of cis-platinum. The current experience of adjuvant treatment after comprehensive staging and cytoreductive surgery reported in the world literature is limited, and the optimal management of the malignancy remains unclear.     

Primary carcinoma of the fallopian tube: study of 11 cases

OBJECTIVE: Primary fallopian tube carcinoma is a rare tumor that histologically and clinically resembles primary ovarian carcinoma. The purpose of this study was to present the experience of the Soroka Medical Center (SMC), Beer-Sheva, Israel of handling this tumor. STUDY DESIGN: Data from the files of 11 patients with primary fallopian tube carcinoma who were managed at the SMC between January 1978 and December 1998 were evaluated. RESULTS: The mean age of the patients was 59.4 years. Presenting symptoms and signs included abdominal pain, postmenopausal bleeding, watery vaginal discharge and adnexal mass. In all patients, the diagnosis of primary fallopian tube carcinoma was not made preoperatively. In ten patients in whom the adnexal mass was discovered preoperatively it was thought to be an ovarian tumor and in one patient the adnexal mass was first noticed during vaginal hysterectomy. Postoperatively, multi-drug chemotherapy was given to seven patients, multi-drug chemotherapy followed by pelvic radiotherapy to one patient, pelvic radiotherapy followed by single-agent chemotherapy to two patients, and one patient received no further treatment. The actuarial 5-year survival rate was 50%. CONCLUSIONS: Fallopian tube carcinoma is rarely suspected preoperatively. The symptom complex of 'hydrops tubae profluence', said to be pathognomonic for this tumor, is rarely encountered. The treatment approach is similar to that used for ovarian carcinoma and includes primary surgery comprised of total abdominal hysterectomy, bilateral salpingo-oophorectomy and staging followed by chemotherapy. The prognosis of patients with primary fallopian tube carcinoma is similar to that of patients with primary ovarian carcinoma.     

Case of peptide-YY-producing strumal carcinoid of the ovary: A case report and review

Ovarian carcinoid is a rare tumor accounting for approximately 0.1% of all ovarian malignancies. We describe a case of peptide-YY-producing strumal carcinoid of the ovary associated with severe constipation. A 48-year-old woman was found to have a pelvic mass on ultrasonography when she visited her primary doctor for a health check-up. She was thus referred to our department. Magnetic resonance imaging revealed a solid right ovarian tumor 60?×?50?mm in size. The patient underwent a right adnexectomy and was histopathologically diagnosed as having strumal carcinoid of the ovary. On immunohistochemical examination, the tumor cells were positive for peptide YY. The patient's constipation resolved rapidly after surgery. Based on her clinical course, her constipation was considered to have been caused by the strumal carcinoid of the ovary. The clinical course of this case supports the previously recognized correlation between peptide-YY-producing ovarian carcinoid and constipation.     

Early isosexual precocious pseudopuberty revealing a juvenile granulosa cell tumor in a six-year-old girl

Juvenile granulosa cell tumors of the ovary are a rare form of neoplasm that makes up less than 5% of ovarian tumors in childhood and adolescence. About 90% are diagnosed in stage I (FIGO) with a favorable prognosis, whereas those at higher stages have a less favorable outcome. The authors describe a juvenile granulosa cell tumor expressed by an early pseudopuberty occurring in a 6-year old child. Clinically, an endocrine syndrome was associated with a pelvic mass. Hyperoestrogenia and serum alphafoetoprotein level were biologically detected. Tumor was localized strictly to the ovary, so conservative surgery was applied and proved sufficient to remove all tumor tissue. Histological examination showed typical microscopic aspect of a juvenile granulosa cell tumor. The patient is well 14 years after surgery with normal growth and mental development.