Fibrothecoma of broad ligament with minor sex cord elements: Case report and brief literature review.

RATIONALE: Sex cord-stromal tumors are always found in ovary, but the occurrence of this kind of tumor at extraovarian locations is extremely rare. Up to now, the case concerning fibrothecoma of broad ligament with minor sex cord elements has not been reported, and it is extremely challenging to diagnose before surgery. In this case report, we summarized pathogenesis, clinical features, laboratory finding, imaging studies, pathology, and therapeutic schedule of this tumor, with the aim of raising awareness and attention to this type of disease. PATIENT CONCERNS: A 45-year-old Chinese woman was referred to our department with intermittent lower abdominal pain for about 6 years. On examination, both ultrasonography and computed tomography revealed she had a right adnexal mass. DIAGNOSIS: Based on the results of histology and immunohistochemistry, the final diagnosis was confirmed as fibrothecoma of broad ligament with minor sex cord elements. INTERVENTIONS: This patient underwent laparoscopic unilateral salpingo-oophorectomy with excision of the neoplasm. OUTCOMES: Eleven days post-treatment, the patient complained that the symptoms of abdominal pain was disappeared. There is no evidence of disease recurrence 5 years after laparoscopic surgery according to the consequences of radiologic examination. CONCLUSION: The natural history of this kind of tumor is uncertain. Although main treatment of this neoplasm might be surgical resection and good prognosis can be achieved, we believe that long-time follow-up is extremely important in all patients diagnosed as fibrothecoma of broad ligament with minor sex cord. Laparoscopic unilateral salpingo-oophorectomy with excision of the tumor should be recommended to these patients.

Giant Ovarian Thecoma Associated with Meigs Syndrome: A Striking Case.

Introduction: Meigs syndrome consists of the presence of a benign ovarian tumor, ascites and pleural effusion, and the latter two subdued after surgical excision of the ovarian tumor. Elevated Ca-125 in this context is confusing and is reported only in a handful of cases. A rare but striking case with the above features is presented herein. Case Presentation: A 46-year-old woman with a giant pelvic/abdominal mass originating from her right adnexa, ascites and pleural effusion, with elevated Ca-125 (938 IU/mL) was treated with the presumptive diagnosis of stage IV ovarian cancer. Imaging modalities showed a 22 cm solid adnexal mass and the patient underwent total abdominal hysterectomy and bilateral salpigooophorectomy, omentectomy and drainage of 4L of ascetic fluid. Surprisingly, final histopathology was negative for malignancy, characterizing the primary tumor as ovarian thecoma. Ascites and pleural effusion resolved by the seventh postoperative day, setting the diagnosis of Meigs syndrome. Discussion: Meigs syndrome accounts for 1% of all ovarian tumors, however it should be considered in the differential diagnosis when clinicians come across the classic triad of the syndrome, even when Ca-125 is elevated. These patients have normal life expectancy with meticulous management, while pathophysiology of this condition remains uncertain in various points.

Ovarian thecoma: A very unusual cause of postmenopausal bleeding.

We describe a 67-year-old woman with postmenopausal bleeding having ceased estrogen plus progestogen therapy nine months before. Transvaginal ultrasonography showed endometrial thickening with normal ovarian appearance. Hormonal studies revealed high estradiol and inhibin B levels but normal androgens and adrenal hormones. Magnetic resonance image demonstrated a 13-mm left ovarian tumour. Hysterectomy and bilateral salpingo-oophorectomy were performed, and the pathological study revealed an 8 mm ovarian thecoma. This case illustrates a very unusual cause of postmenopausal bleeding. We suggest a study protocol and discuss the differential diagnosis of this case.

COMPLETE REMISSION OF MEDICALLY TREATED LUTEINIZED THECOMAS WITH SCLEROSING PERITONITIS.

We describe the first French patient treated for sclerosing peritonitis syndrome associated with lutheinized thecomas. At 42 years old, she had respiratory distress with increased abdominal volume. Physical examination revealed ascites, pleural effusions, and two mobile latero-uterine masses. Radiological examinations revealed bilateral ovarian masses of 10 cm. Bilateral adnexectomy was performed by laparotomy. Histological analysis concluded that there were benign luteinized thecomas. Until the 36th postoperative day, the general condition of the patient deteriorated to become critical. A second surgical procedure was attempted revealing sclerosing fibrosis preventing access to the peritoneal cavity. Subsequently, a medical treatment combining parenteral nutrition, high intravenous doses of corticosteroids, antiestrogens, colchicine and sandostatin was administered and effective allowing continuity recovery 15 months later. The clinical outcomes has been favorable at 24 month later.

Adolescent ovarian thecoma presenting as progressive hyperandrogenism: case report and review of the literature.

Hyperandrogenism is frequent and under investigated in adolescent girls. A 15-year-6-month-old French girl presented with oligomenorrhea and slowly progressing virilization 2 years post-menarche. Medical history revealed prenatal pesticide exposure through maternal professional activity and recurrent premature thelarche. Severe hirsutism, mild facial acne and clitoromegaly were noted. Serum androgens (testosterone: 94 ng/dL, 4-androstenedione: 8.23 ng/mL) were high and non-classic 21-hydroxylase deficiency was excluded. Pelvic ultrasonography showed a left ovarian mass, confirmed by computed tomography scan. Tumor markers were negative. Laparoscopic surgery was performed. The pathological diagnosis was benign luteinized thecoma. Postoperatively, the menstrual cycle and serum androgens became normal and hirsutism slowly improved. Hyperandrogenism 2 years after menarche should be systematically investigated, even if slowly progressive, since it may be a symptom of a rare virilizing ovarian tumor, like thecoma.

High cellularity and mitotic activity in a primary ovarian fibro-thecomatous tumor of a young patient: a diagnostic and clinical challenge.

Purpose ofInvestigation: Solid ovarian tumors represent a clinical challenge, in particular in case of young patients who require a fertility sparing treatment. The authors report a case of hypercellular mitotically active ovarian fibrothecoma in a very young woman, successfully treated with a fertility sparing surgery. MATERIALS AND METHODS: A 21-year-old nulliparous woman presented at the present hospital with a 14-cm right ovarian mass, consisting of solid and pseudo-cystic components. There was neither an elevation of tumor markers nor evidence of metastatic disease. A laparotomic right salpingo-oophorectomy was performed. Uterus and left adnexa were preserved. RESULTS: The neoplasm consisted of a prevalent population of spindle-shaped elements and of a minor component of cells with wider cytoplasms, attributable to a thecomatous differentiation. The mitotic activity was focally elevated. Cytological atypia was mild to focally moderate. Clear areas of coagulative necrosis were not observed. At present 48 months after surgery, the patient is alive with no evidence of recurrence. CONCLUSIONS: The authors reported the lesion as a hypercellular and mitotically active fibrothecoma. The uneventful follow-up confirms the low malignant potential of the lesion. Caution is required reporting hypercellular stromal ovarian tu- mors, in order to avoid overdiagnosis and overtreatment, particularly in young patients.

Vestibulodynia: A Multifactorial Syndrome: A Case Report.

BACKGROUND: Vulvodynia is a difficult-to-treat, chronic, multifactorial malady that drastically lowers the quality of life of afflicted patients. CASE: A 68-year-old woman, who had been treated successfully for vulvodynia years before with medication, returned with a recurrence of vulvodynia symptoms that this time did not respond to treatment. She now had biopsy-confirmed lichen sclerosis and was found to have markedly elevated serum testosterone levels. An imaging study detected an ovarian lesion that, on removal, proved to be afibrothecoma. Postoperatively the testosterone rapidly dropped to normal levels. What was unexpected and unusual was that the vulvar pain disappeared and the lichen sclerosis markedly regressed. CONCLUSION: This case demonstrates a hormonal trigger for the development of vulvodynia.

Luteinized Thecomas ("Thecomatosis") with Sclerosing Peritonitis (LTSP): Report of 2 Cases and Review of an Enigmatic Syndrome Associated with a Peritoneal Proliferation of Specialized (vimentin+/keratin+/CD34+) Submesothelial Fibroblasts.

OBJECTIVE: To present the clinicopathologic features of two cases of luteinized thecomas with sclerosing peritonitis (LTSP), characterize the cellular proliferation in the sclerosing peritonitis (SP), and review the literature. METHODS: The clinical, laboratory, and imaging data, operative findings, and pathology materials were reviewed and summarized. Samples of the SP were stained with keratin AE1/AE3, vimentin, CD34, calretinin, smooth muscle actin, ER/PR, CD10 and desmin. A literature search was performed to identify cases of LTSP for comparison. RESULTS: A total of 43 cases of LTSP syndrome were identified. Frequent clinical features included ascites (74%), abdominal pain (35%), bowel obstruction (42%), and bilateral masses (84%). We isolated a distinct form of ovarian luteinized thecoma (thecomatosis) and peculiar sclerosing peritonitis (SP). IHC analysis shows a proliferation of specialized (vimentin+/keratin+/CD34+) submesothelial fibroblasts (SMF) with patchy expression of calretinin and hormone receptors. CONCLUSION: LTSP syndrome is a rare entity presenting with abdominal pain, bowel obstruction, ascites, ovarian masses, and SP containing specialized (vimentin+/keratin+/CD34+) SMF. LTSP must be distinguished from abdominal cocoon, isolated SP, Meigs' syndrome, and peritoneal carcinomatosis. The importance of recognizing the diagnosis is stressed, as failure to manage this disease conservatively leads to significant morbidity and mortality. The SP and bowel obstruction may persist for months, even after resection of the tumours, resulting in extended medical therapy. Based on the immunophenotype of the peritoneal lesions, strategies to elucidate 'targeted' pharmacologic agents that could inhibit the proliferation of specialized (vimentin+/keratin+/CD34+) SMF may be of benefit.

Unilateral ovarian fibrothecoma with menorrhagia.

Ovarian fibrothecoma is a relatively new term that is used to describe an ovarian sex cord stromal tumour that has mixed features of both fibroma and thecoma. The prevalence of ovarian fibrothecoma tumours is very rare and is reported to be about 1.2% of all ovarian tumours. We report a case of a 32-year-old woman who presented with acute menorrhagia with no previous medical, surgical or gynecological history. She was amenorrhic for four years after the insertion of a levonorgestrelreleasing intrauterine system (LNG-IUS) for contraception. The efficacy and location of LNG-IUS was reflected due to the sudden onset of menorrhagia. On pelvic examination and ultrasound the LNG-IUS could not be visualized and a uterine fibroid was noted. A diagnostic laparoscopy was done to identify the LNG-IUS, which revealed an incidental large ovarian mass on the left ovary. CA-125 level was elevated to 45 kU/L (Normal range <35 kU/L). Total abdominal hysterectomy, left salpingo-oopherectomy and cystectomy were performed. On histopathology, the mass was proven to be an ovarian fibrothecoma. No signs of malignancy were noted on peritoneal fluid cytology. The LNG-IUS was found inside the uterus. Our case is reported on the basis of the rare incidence of ovarian fibrothecoma and the possible effect it may have on the efficacy of LNG-IUS causing menorrhagia.

Recurrent femoral hernia and associated ovarian pathology.

The following case describes an ovarian tumour presenting in a highly unusual manner-in the form of a recurrent femoral hernia. Recurrent femoral herniae are unusual and should prompt awareness of underlying pathology causing increased intra-abdominal pressure.

[A woman with an increasing belly size in spite of diet]. / Een vrouw met toegenomen buikomvang ondanks dieet.

A 44-year old women presented with a increasing abdomen and mild pain since several months. In surgery a cystic fibrothecoma of the ovary of 10 kg was removed. Fibrothecoma's are rare benign tumours of the ovaries. These tumours rarely grow this big.

Mitotically active cellular luteinized thecoma of the ovary and luteinized thecomatosis associated with sclerosing peritonitis: case studies, comparison, and review of the literature.

In this study, we distinguish two clinical and pathological entities that are similarly named: luteinized thecoma and luteinized thecoma associated with sclerosing peritonitis. Ovarian luteinized thecoma lacks definitive criteria for malignancy. Based on our case study of a mitotically active neoplasm without nuclear atypia in which the patient was living and well 19 years after operation and comparison with prior studies of luteinized thecoma and the closely related entity of cellular fibroma, we propose presumptive criteria for malignancy for this rare neoplasm. Increased mitotic activity in luteinized thecoma without significant nuclear atypia is not an indication of malignant behavior, and such cases should therefore be referred to as mitotically active cellular luteinized thecoma. We also contrast neoplasms in the luteinized thecoma category with the entity originally reported as luteinized thecoma associated with sclerosing peritonitis. In the latter, the ovarian stromal proliferations are typically bilateral, can have an exceedingly high mitotic rate as was seen in our illustrative case, often incorporate non-neoplastic ovarian structures at their periphery, and are responsive to medical therapy. In our patient with sclerosing peritonitis, both the ovarian masses and peritoneal sclerosis underwent complete regression following treatment with gonadotropin-releasing hormone agonist and high doses of steroids, and an ovarian biopsy taken 2 months after therapy showed a histologically normal ovary. The patient subsequently became pregnant and delivered a normal infant. This is, to our knowledge, the first case of successful medically conservative treatment of a young patient with this entity that led to complete relief of symptoms and allowed preservation of fertility. Because recent observations support the non-neoplastic nature of the ovarian stromal proliferations, we advocate use of the previously proposed term luteinized thecomatosis associated with sclerosing peritonitis for this entity.

Juvenile granulosa and theca cell tumor of the ovary as a rare cause of precocious puberty: case report and review of literature.

BACKGROUND: The differential diagnosis for precocious puberty in a young female includes peripheral causes. This case documents a rare cause of peripheral precocious puberty--a juvenile granulosa and theca cell ovarian tumor--and a brief review of the literature for this tumor type. CASE: A 7-year-old girl presented with rapid onset of pubertal development and elevated estradiol levels. Menarche occurred 5 months after thelarche. A thorough workup revealed a large multicystic left ovary. Other causes of precocious puberty were excluded. She underwent an exploratory laparotomy and left salpingo-oophorectomy. Pathology reported a juvenile granulosa and theca cell tumor of the ovary, FIGO stage 1A. Postoperatively, she experienced a cessation of vaginal bleeding and estradiol levels normalized. A literature review found that early stage disease has an excellent prognosis and that adjuvant chemotherapy is not indicated in this setting. SUMMARY AND CONCLUSION: Juvenile granulosa and theca cell tumor of the ovary is a rare cause of peripheral precocious puberty, even more so than juvenile granulosa cell tumor, due to the theca component. Treatment is surgical and an excellent prognosis is possible for early stage disease.

Testicular thecoma in an 11-year-old boy with nevoid basal-cell carcinoma syndrome (Gorlin syndrome).

We report a case of testicular thecoma in an 11-year-old Japanese boy with nevoid basal-cell carcinoma syndrome (Gorlin syndrome). He presented with left testicular swelling and underwent a radical orchiectomy on suspicion of a malignant paratesticular tumor. The tumor arose from the testis exophytically and was diagnosed as a thecoma histopathologically. Ovarian thecoma-fibroma group tumors are closely associated with Gorlin syndrome or with abnormalities in PTCH, a candidate gene for the syndrome. The occurrence of an extremely rare testicular thecoma in this case (the second in the literature) suggests that such an etiological association may also exist in the pathogenesis of testicular tumors.

Isolated luteinizing hormone (LH) elevation in a woman with secondary amenorrhea: a clue to the diagnosis of an inhibin B-producing thecoma and insights into the influence of inhibin B on LH.

OBJECTIVE: To review the diagnostic possibilities that exists when the workup of amenorrhea reveals an isolated LH elevation; and to examine the effect of inhibin B on LH levels in vivo. DESIGN: Case report. SETTING: University hospital. PATIENT(S): A 20-year-old woman presented with secondary amenorrhea. Her FSH measurement was low, and the LH level was elevated. The recognition that this was an unusual pattern led to the diagnosis of a rare but very treatable inhibin B-producing thecoma, despite the fact that results on the initial pelvic ultrasound examination performed 10 months after presentation of amenorrhea were relatively unremarkable. INTERVENTION(S): Surgical removal of an ovarian thecoma. MAIN OUTCOME MEASURE(S): Gonadotropins, E2, inhibin B, menstrual bleeding, and fertility. RESULT(S): Removal of the ovarian thecoma resulted in a normalization of FSH, LH, and inhibin B levels and a return of spontaneous menses 28 days later. Pregnancy occurred with the third postoperative menstrual cycle, followed by the delivery of a healthy full-term girl. CONCLUSION(S): Inhibin B-producing sex cord granolosa-stromal cell tumors should be considered in women who present with amenorrhea with isolated LH elevations, even in the setting of a previously normal pelvic ultrasound report. Diagnostic considerations that arise in the workup of amenorrhea when there is an isolated elevation in LH that is accompanied by normal or low FSH levels are reviewed. This rare clinical presentation provides the opportunity to observe the impact of inhibin B on gonadotropins in vivo.

Ossifying luteinized thecoma of the ovary with endometrial adenocarcinoma.

Luteinized thecomas are one of the sex cord stromal tumors of the ovary rarely undergoing ossification. We report a case of a 66-year-old post-menopausal female with the chief complaint of uterine bleeding of 7 months duration. Endometrial curettage performed showed features of endometrial adenocarcinoma. Follow-up total abdominal hysterectomy revealed bilateral luteinized thecomas of the ovary, one of which had undergone massive ossification converting the ovary into a bone. True bone formation in ovarian tumors is rare. This case is the second in the literature of osseous metaplasia in an ovarian luteinized thecoma, with the association of endometrial adenocarcinoma suggesting its functional status.