Fatal case of luteinized thecoma with sclerosing peritonitis in a 40-year-old woman.

We describe the pathologic and clinical presentation of a very rare, fatal case of luteinized thecoma with sclerosing peritonitis in a 40-year-old woman, who had a history of total abdominal hysterectomy and a left salpingo-oophorectomy in 2003. The patient presented with abdominal pain, and radiologic examinations revealed a 10-cm heterogenous right pelvic mass with partial necrosis. The patient eventually underwent an exploratory laparotomy, which revealed an ovarian tumor with multiple implants in the peritoneal cavity. The ovarian lesion was made up of spindle cells among clusters of luteinized stromal cells that expanded to the ovarian cortex. Tumor cells were positive for vimentin, estrogen and progesterone receptors, and CCD68 (focally) and negative for CD34, α-smooth muscle actin, ß-catenin, and desmin by immunohistochemical studies. Luteinized cells were positive for α-inhibin and calretinin. Tumor cells exhibited low Ki-67 proliferation indices. The patient died because of the sclerosing peritonitis component of the disease.

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.

[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.

Review of the granulosa-theca cell tumors from the emil Novak ovarian tumor registry.

OBJECTIVE: Our purpose was to review patients with granulosa and theca cell tumors as filed in the Emil Novak Ovarian Tumor Registry. STUDY DESIGN: Our study was a descriptive, retrospective study of 454 case records. RESULTS: The reviewed diagnoses were for 97 patients with granulosa cell tumors, 116 with theca cell tumors, and 97 with granulosa-theca cell tumors. The remaining cases (n = 144) were reclassified as "nonspecific" gonadal stromal tumors (n = 61), luteomas of pregnancy (n = 7), and 76 "other" cases. These included poorly differentiated cancer, metastatic cancer, mixed mesodermal tumors, and sarcomas. The tumor-related mortality rate for the 310 patients with granulosa, theca, and granulosa-theca cell tumors was 7% (37.3% for granulosa cell tumors only). The surgical stage of disease was the most significant prognostic factor, with a mortality rate of at least 40%, given that the tumor had spread beyond the ovary. CONCLUSION: Because the differential diagnoses of particularly granulosa cell tumors included several conditions with an extremely poor prognosis, an accurate histologic diagnosis is crucial.

Granulosa and theca cell tumors in children: a report of 17 cases and literature review.

The files of the Emil Novak Ovarian Tumor Registry (ENOTR) were searched for granulosa and theca cell tumors in children aged 12 years and less. In addition, an extensive literature search was done for English publications on children with these tumors aged 10 years or less. Of the 17 children from the ENOTR, 5 had adult-type granulosa cell tumors, 6 had juvenile granulosa cell tumors, and 1 had a luteinized granulosa cell tumor. In addition, there were three cases with gonadal stomal tumors, one theca cell tumor, and one granulosa-theca cell tumor. Precocious pseudopuberty was present in 70 percent of the children, abdominal pain in 24 percent, and ascites in 18 percent. The literature review revealed a tumor-related mortality rate of 9 percent (based on 163 cases with granulosa cell tumors, including the juvenile type). Some of these tumors are large with acute pain, but nevertheless, the prognosis is good, particularly in cases with precocious puberty. Treatment can be conservative.

[The prognostic factors in patients with malignant granulosa-theca cell tumors of the ovaries]. / Faktory prognoza u bol'nykh so zlokachestvennymi granulezotekakletochnymi opukholiami iaichnikov.

The extent of tumor, as revealed by surgery in 113 primary patients with malignant granulosa-stromal cell tumors of the ovaries (granulosa cell--77, theca cell--17 and mixed theca-granulosa cell tumors--19 patients), was analysed versus duration of disease, laterality of tumor and patient age. Factors which were significantly or as a marked tendency associated with stage III and IV of disease and thus suggested poor prognosis were identified.

Survival after ovarian granulosa and theca cell tumours.

Ninety-two cases of ovarian granulosa and theca cell tumours were recorded in the Tayside Gynaecological Tumour Registry during the 30 year period 1948-78. A high follow-up rate was achieved through this centralised registry and exhaustive annual recall. At 20 years after initial treatment patient actuarial survival was 92% for the theca cell tumours and 34% for granulosa cell tumours.

[Malignancy of hormonally active ovarian tumors]. / K voprosu o zlokachestvennosti gormon-aktivnykh opukholei iaichnika.

401 cases of hormone-producing tumors of the ovary were studied (190 theca-cell, 169--granulose cell, 25--mixed thecagranulosa cell and 17--virilising tumors). Histological diagnosis, clinical stage and end results of their treatment were compared. Theca-granulosa cell tumor is considered to be the most malignant form of these neoplasms. Pure theca-cell tumor proved to be benign in most cases. Of special interest is a high percentage of patients being cured at stage 1 (FIGO classification) of neoplastic process, that stands, in the authors' opinion, in close relationship with comparatively good results obtained in these patients.

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.

Granulosa-cell and theca-cell tumors. The clinical picture and long-term outcome for the Radiumhemmet series.

Over the period 1923--72 a total of 305 patients with granulosa-cell, theca-cell tumor and mixed tumor were seen at Radiumhemmet. The commonest symptom was abnormal uterine bleeding. Twenty-three per cent of married women were nulliparous. The menarche seems to have occurred earlier in this group than in the general population. Eight of the granulosa-cell tumor group gave birth to 12 children after treatment for the tumor. Nineteen women with granulosa-cell tumor and 4 with thecoma had received radiotherapy earlier in life for benign lesions. The risk of endometrial cancer was approximately 10 times greater for the women with granulosa- or theca-cell tumor than for the general population. Two hundred and fifty-two patients were given both surgical treatment and radiotherapy. Fifty-three received only surgery (37 granulosa-cell and 16 theca-cell tumors). None of the thecoma patients but 21 per cent of those with granulosa-cell tumor died from their disease. The 5-year survival for the latter group, all stages, was 85 per cent.