Luteinized thecoma (thecomatosis) with sclerosing peritonitis: a systematic review of the literature of the last 25 years.

INTRODUCTION: Luteinized thecoma (thecomatosis) with sclerosing peritonitis (LTSP) is a very uncommon syndrome, characterized by the presence of single or bilateral ovarian thecomas and peritoneal fibrotic lesions. The disease occurs in young women and it can lead to peritoneal fibrosis and bowel obstruction. The pathogenesis of this syndrome remains still largely unknown. Surgery represents the cornerstone of treatment, but resection alone does not always allow a complete disease control. Attempts at medical treatments have been reported in recent years, but a real standard therapy has not yet been defined. AREAS COVERED: We performed a systematic review of literature, collecting all the papers that reported cases of LTSP, since its first description in 1994. We found that, in these 25 years, less than 50 cases have been described in literature. EXPERT OPINION: Along with the established role of surgery, adjuvant treatment with hormonal agents, in particular in estrogen receptor expression, seems to be a promising approach. However, more efforts must be carried out to describe treatment and outcome of new cases, improving knowledge about this rare condition.

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.

Testicular fibrothecoma: a morphologic and immunohistochemical study of 16 cases.

Benign intratesticular spindle cell lesions are rare. Herein, we report the morphology, immunohistochemical characteristics, and prognosis of 16 cases of testicular fibrothecoma. The mean age at diagnosis was 44 years (16 to 69 y). Of 15 patients with information, 14 presented with a palpable testicular mass and 1 with heaviness in the scrotum. Medical histories included bilateral orchidopexy as a child (n=1) and testicular atrophy receiving testosterone replacement (n=1). The average size was 1.8 cm (median 2 cm; range, 0.5 to 7.6 cm). All cases were intratesticular, although 13 were abutting the tunica albuginea with others centered on the rete testis (n=2), or were indeterminate on biopsy (n=1). Eleven cases were relatively well circumscribed, although not encapsulated, with 1 being infiltrative and 4 not evaluable. Four tumors showed entrapment of seminiferous tubules. Half of the fibrothecomas showed a mixed storiform pattern and short fascicles, with 6 storiform only and 2 short fascicles only. One half of the tumors were very hypercellular. Cases were equally split between having plumper ovoid as opposed to spindled pointed nuclei, with all cases lacking prominent nucleoli. Eleven cases had 0 to 2 mitoses per 10 HPF, 3 had 4 to 5 mitoses per 10 HPF, and 2 had 9 to 10 mitoses per 10 HPF. Collagen deposition either in bands or investing single cells ranged from none to extensive, with 10/16 cases having at least a moderate amount. Immunohistochemical positivity was as follows: inhibin (11/13, patchy to diffuse); calretinin (5/9); Melan-A (4/4); pan keratin (5/8); BCL2 (3/4); CD34 (3/8); S100 (4/8); muscle-specific actin (4/4); and desmin (5/8). Patients were followed up for a mean of 71.8 months (range, 3 to 144 mo). All were well with no evidence of disease. Of the 2 men with 9 to 10 mitoses per 10 HPF, 1 died of other causes 5 years and 8 months later, and the other had no evidence of disease at 4 years and 10 months after surgery. In summary, testicular fibrothecomas are rare with somewhat variable histology and can have worrisome histologic features such as minimal invasion into surrounding testis, high cellularity, and increased mitotic rate. Their immunoprofile is variable and typically not diagnostic. Despite some worrisome histologic features, they appear uniformly benign in their behavior.

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.

Incidence, clinical analysis, and management of ovarian fibromas and fibrothecomas.

OBJECTIVE: The objective of the study was to identify the incidence, diagnosis, and therapeutic and histological particularities of ovarian fibromas and fibrothecomas. STUDY DESIGN: This was a retrospective study of 24 patients who underwent surgical treatment for ovarian fibromas and fibrothecomas between January 1994 and December 2006. Clinical, ultrasonographic, tumor marker, therapeutic, and histologic data were analyzed. RESULTS: The mean age of patients was 49 years. Thirteen patients were menopausal. Ultrasonographic findings were ovarian echogenic tumor in 6 cases, hypoechogenic tumor in 12 cases, mixed tumor in 6 cases, and anechogenic tumor in 1 case. Cancer antigen-125 level measured in 21 cases was abnormal in 3 cases. Twenty-one patients underwent laparotomy. Three patients underwent laparoscopy; however, 1 was converted to laparotomy because of a suspected tumor. Histological findings were 16 fibromas and 9 fibrothecomas. CONCLUSION: Ovarian fibromas and fibrothecomas are uncommon, accounting for 3.3% of ovarian tumors. These lesions often occur in perimenopausal and menopausal patients. Clinical, ultrasonographic, and tumor marker data remain the best preoperative approach currently available for ovarian tumors. However, the diagnosis remains histological.

Luteinized thecoma associated with sclerosing peritonitis--conservative surgical approach followed by corticosteroid and GnRH agonist treatment--a case report.

BACKGROUND: Luteinized thecoma of the ovary associated with sclerosing peritonitis is a rare tumor that has no standard definitive treatment regimen. CASE: A 25 year-old patient diagnosed with luteinized thecoma and sclerosing peritonitis in the omentum. The patient received high dose corticosteroids (IV Hydrocortisone 500 mg/d) and GnRH agonist (IM Leuprolide 3.75 mg) in order to achieve ovarian suppression and relief of the clinical peritonitis. She was re-admitted two weeks later due to bowel obstruction which was treated conservatively. The steroid regimen was continued by oral intake for 5 weeks with complete remission of the peritonitis related symptoms. The bilateral enlarged ovarian tumor-like solid was the prominent finding in consecutive ultrasound exams with no decrease in size despite of the above mentioned protocol. Thus, the patient was re-operated for exploration and biopsies of the ovary and the pathology report showed no evidence of remnant disease in the ovary, or in the peritoneum. Completing follow-up of 15 months since the last operation, the patient is asymptomatic. She conceived spontaneously and currently is in her 24th week of a normal pregnancy. CONCLUSION: This is the first case report in the English literature of a successful medical conservative treatment of a young patient with luteinized thecoma associated with sclerosing peritonitis that led to complete relief of the symptoms and allowed fertility preservation.

[Clinical analysis of 74 cases with ovarian thecoma].

OBJECTIVE: To study the clinical pathologic characteristics, treatment and prognostic factors of ovarian thecoma. METHODS: From 1958 approximately 1998, a total of 74 patients with benign and malignant thecoma were retrospectively analyzed. RESULTS: The reviewed diagnosis were 8 patients with malignant thecoma, 66 patients were benign thecoma. Nine of 66 patients with benign thecoma appeared cell proliferative activity. The mean age was 52 years. The most frequent symptoms were abdominal-pelvic masses (56.8%), metromenorrhagia and abnormal vaginal bleeding, other symptoms were abdominal pain, infertility followed. Twenty-two of 66 patients had ascites, only 1 patient with ascites and hydrothorax simultaneously. Thirty-nine patients with diseases which associate with excessive estrogenic stimulation, including 1 endometrial adenocarcinoma and 1 endometrial dysplasia and other diseases such as: myoma; endometrial hyperplasia and polyp. Fifteen patients with benign thecoma were evaluated blood serum CA(125), the CA(125) were elevated in 9 of 15 patients. In 2 of 9 patients with cell proliferative activity, the tumors invaded adjacent tissues or relapsed, after received operation and pelvic radiotherapy, they are alive 11 years and 27 years respectively. Among 8 patients with malignant thecoma, 4 patients without postoperation therapy or with non-standard chemotherapy died in 2 years postoperation, the other 4 patients received operation, postoperational radiotherapy or (and) chemotherapy, 1 of 4 patients died of irradiation intestinal fistule 4 years later; two patients are alive without disease more than 10 years, another one for 2 years. CONCLUSIONS: Theca cell tumor of ovary have good prognosis, but we should pay attention to thecomas with proliferative activity. The prognosis of malignant thecomas is poor, postoperative systemic chemotherapy or radiotherapy to the patients with malignant thecomas can improve their survival.

[Ovarian fibrothecal tumors. Apropos of 12 cases]. / Les tumeurs fibrothécales de l'ovaire. A propos de 12 observations.

OBJECTIVE: To identify diagnosis, therapeutic and histological particularities of ovarian fibro-thecomas. STUDY DESIGN: A retrospective study of 12 patients who underwent surgical treatment for ovarian fibro-thecoma between January 1994 and December 1999 was reported. Clinical, ultRasonographic, tumour marker, therapeutic and histologic data were analysed. RESULTS: Mean age of patients was 46.83 years. Three patients were in perimenopausal period and six were menopausal. Ultrasonographic findings were: ovarian echogenic tumor in six cases, hypoechogenic tumor in three cases, mixed tumor in three cases and anechogenic tumour in one case. CA 125 level measured in 11 cases was normal. Nine patients underwent laparotomy. Three patients underwent laparoscopy, however, one was converted to laparotomy due to a suspected tumour. A conservative treatment was performed for three patients and radical treatment for nine patients. Histological findings were eight fibromas and five fibro-thecomas. CONCLUSION: Ovarian fibro-thecoma is uncommon. These lesions occur often in perimenopausal and menopausal patients. Clinical, ultrasonographic and tumour marker data remains the best preoperative approach currently available for ovarian tumours. However, the diagnosis remains histological. Tumorectomy is well indicated for young patients, however radical treatment is indicated for perimenopausal and menopausal patients.

Secondary amenorrhea and infertility caused by an inhibin-B-producing ovarian fibrothecoma.

OBJECTIVE: To report a case of secondary amenorrhea and infertility caused by an inhibin-B-producing ovarian fibrothecoma. DESIGN: Case report. SETTING: Academic medical center. PATIENT: A 37-year-old woman with a 2-year history of secondary amenorrhea and infertility. INTERVENTION(S): Operative removal of a 5-cm ovarian fibrothecoma. MAIN OUTCOME MEASURE(S): Luteinizing hormone, FSH, E2, inhibin-B, TSH, and prolactin measured preoperatively and postoperatively. Immunostaining of tumor cells for inhibin and LH. RESULT(S): Preoperative hormone levels were as follows: FSH, 1.7 mIU/mL; LH, 23.4 mIU/mL; E2, 31 pg/mL; and inhibin B, 1,154 pg/mL. Three weeks postoperatively, the FSH was 1.5 mIU/mL, LH decreased to 7.1 mIU/mL, E2 increased to 276 pg/mL, and inhibin-B decreased to 17 pg/mL. The fibrothecoma did not stain for LH but was strongly positive for inhibin. Regular menstrual cycles resumed 28 days postoperatively. CONCLUSION(S): Inhibin-B produced by an ovarian tumor profoundly suppressed FSH levels and resulted in secondary amenorrhea and infertility. Use of sensitive and specific immunoassays for inhibin-A and -B may aid in the differential diagnosis of hormonally active ovarian tumors.

Management of non-epithelial ovarian tumours.

Ovarian tumours of non-epithelial origin are less common than those of epithelial origin but must be distinguished from these as their natural history and management differ. As these tumours are rare, histological review by an expert in the field is essential. There have been no randomised trials to outline the management of these tumours and therefore this paper represents a review of descriptive data. Non-epithelial ovarian tumours, which represent approximately 50% of all ovarian tumours and approximately 25% of malignant ovarian tumours, are outlined. Lymphomas and metastatic tumours should be treated the same as when found in other sites and will not be discussed further.

Diagnosis and management of bilateral theca lutein cysts in a normal term pregnancy.

Theca lutein cysts are benign neoplasms known to be associated with twins, molar pregnancy and erythroblastosis fetalis, but they are only rarely associated with a normal singleton pregnancy. Their natural course is postpartum spontaneous regression. Documented cases were noted because of abdominal pain or dystocia. This paper reports theca lutein cysts which were asymptomatic and were first noted ultrasonographically in a singleton gestation at term. In a few published cases and in a larger number of unpublished cases, apparent confusion as to the benign nature of these cysts has led to unwarranted bilateral oophorectomy. Asymptomatic theca lutein cysts undoubtably occur more commonly than they are reported. With the increasing use of ultrasonography and cesarean section, this condition probably will be observed more frequently in the future. At the time of laparotomy in late pregnancy with ovaries which appear pathological, tissue biopsy and evaluation is of critical importance in order to avoid unnecessary castration in this group of young women.

Clinicopathologic review of 118 granulosa and 82 theca cell tumors.

The clinical course and histologic features of 118 granulosa cell tumors and 82 theca cell tumors were reviewed. Although the 2 cell types are related, important differences exist in their behavior. Theca cell tumors (TCTs) are virtually benign, while granulosa cell tumors (GCTs) exhibit less malignant activity than most ovarian carcinomas. Characteristically detected at an early stage, GCTs may recur locally years after the initial diagnosis. Survival figures depend on tumor stage. Recurrence of GCTs is associated with a high mortality rate. The cornerstone of treatment is total abdominal hysterectomy and bilateral salpingo-oophorectomy, regardless of age. Adjuvant radiation or chemotherapy may be helpful when lesions are higher than stage lai. Therapy for recurrence usually requires operative excision combined with radiation, chemotherapy, or both.

Theca-cell tumors. Clinical features and prognosis.

During the period 1923 to 1972, 62 women with theca-cell tumors were treated at Radiumhemmet. The mean age at diagnosis was 59.5 years. Concomitant endometrial carcinoma was found in 13 patients. At 1978, after a mean observation time of 15.5 years, 30 patients were alive. No patient died from thecoma. Malignant disease caused death in 7 women. No difference in survival was found between 20 patients and 40 controls matched by age and place of residence.