Reports on neoplasms in bears are scarce, especially concerning ovarian tumors. A large primary ovarian neoplasm with multiple metastasis was found during the necropsy of a 14-year-old free-ranging Eurasian brown bear (Ursus arctos) from Northwestern Spain. Histopathology and immunohistochemistry allowed for the diagnosis of a sex cord stromal tumor. This is a complex group of neoplasms which differ in the predominant cell morphology and immunohistochemical features. The microscopic examination revealed two types of cells, one with eosinophilic cytoplasm, intermingled with larger vacuolated cells rich in lipids. The evaluation of the immunoreactivity to different markers, frequently used in the characterization of gonadal tumors (INHA, inhibin-alpha; PLAP, placental alkaline phosphatase; Ki-67; α-SMA, actin alpha-smooth muscle) and inflammation patterns (IBA1, ionized calcium-binding adapter molecule for macrophages; CD3 for T lymphocytes; CD20 for B lymphocytes), displayed significant INHA positive immunostaining of neoplastic cells, as well as inflammatory cell infiltration, mainly composed of macrophages and B lymphocytes. These findings were consistent with a malignant ovarian steroid cell tumor, not otherwise specified. The present study characterizes an unusual type of neoplasm, and also represents the first report of an ovarian sex cord stromal tumor in Ursidae.

Microscopic heterotopic extraovarian sex cord-stromal proliferations were first reported in the literature in 2015 by McCluggege. Afterwards, few similar cases have been described. Herein, we report the fourteenth case of microscopic heterotopic sex cord-stromal proliferation and the third case sited in the pelvic peritoneum. The clinical history of these rare cases suggests their benign nature. Knowledge of this histological pattern is important for differential diagnoses such as malignant pathologies and metastatic diseases.

Steroid cell tumors (SCT) of the ovary are rare, which has limited advances in the understanding of this enigmatic neoplasm. In this review, we summarize currently known clinicopathologic information on SCT. SCT are frequently hormonally active, leading to elevated serum and/or urine levels of androgenic hormones or their metabolites, and associated symptomatology, including virilization. The reported age at diagnosis is broad and has ranged from as young as 1 year old to 93 years old, although most patients were between ages 20 and 40 years. Most tumors are stage I and unilateral. The tumors are usually well circumscribed with a solid or solid to cystic cut surface. The tumors in one series reportedly ranged in size from 1.2 to 45 cm (average 8.4 cm). MRI is a useful imaging modality, typically showing a well delineated mass with contrast enhancement and lipid content on T2 and T1 weighted images, respectively. Microscopically, SCT display polygonal to epithelioid cells with abundant eosinophilic to vacuolated/clear cytoplasm and display an immunoprofile that is consistent with sex cord-stromal differentiation. Most cases are benign, without any recurrences after primary resection, but a subset - probably less than 20% of cases -are clinically malignant. Pathologic criteria that can specifically predict patient outcomes remain elusive, although features that correlate with adverse outcomes have been proposed based on retrospective studies. The molecular characteristics of SCTs are similarly under characterized, although there is some evidence of an enrichment for hypoxia-signaling gene mutations in SCT. In malignant SCT, the tumors generally show greater global genomic instability, copy number gains in oncogenes, and occasional BAP1 mutation. Future studies involving multi-institutional cohort and unbiased molecular profiling using whole exome/transcriptome sequencing are needed to help advance our molecular understanding of SCTs.

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Ovarian cancer is among the most common types of cancer suffered by the female population. As of United States Cancer Statistics (USCS) 2019, the National Cancer Institute reports the prevalence of ovarian cancer as 11.4 cases per every 100,000 each year. The highest prevalence is in the seventh decade of life. Of all the types, sex cord-stromal tumors (SCSTs) account for 5-8% of cases. They are a heterogeneous group of rare neoplasms originating from the ovarian matrix, and nearly 90% of the hormone-producing tumors are SCSTs. Hence, patients with SCSTs are known to present with excess estrogen and androgen signs and symptoms. Many SCSTs are known for their indolent course and tendency to affect the unilateral ovary. The prognosis of the malignancy depends on the subtype of SCST, the stage of the patient\'s disease, and age. Among all the types, 20-50% of the ovaries\' granulosa cell tumors tend to recur decades after the initial presentation, and 70% of the recurrences end up with a very poor prognosis. This case will discuss a 68-year-old woman who presented with a recurrence of an adult granulosa cell tumor after 13 years in remission. The patient had been previously diagnosed with an adult granulosa cell tumor of the right ovary at age 55 and had undergone surgical resection along with chemotherapy.

Identifying prognostic factors and evaluating the impact of adjuvant chemotherapy in patients with sex cord stromal tumors (SCST) is crucial. In this study, we aimed to address these challenges.
We conducted a retrospective analysis of data from 13 centers of the French Rare malignant gynecological tumors (TMRG) network. We enrolled 469 adult patients with malignant SCST who received upfront surgery since 2011 to July 2015.
75% were diagnosed with adult Granulosa cell tumors, and 23% had another subtype. With a median follow-up of 6.4 years, 154 patients (33%) developed a first recurrence, 82 (17%) two recurrences, and 49 (10%) three recurrences. Adjuvant chemotherapy was administered in 14.7% of patients at initial diagnosis. In relapse, perioperative chemotherapy was administered in 58.5%, 28.2%, and 23.8% of patients, respectively, in the first, second, and third relapse. In the first-line therapy, age under 70 years, FIGO stage, and complete surgery were associated with longer progression-free survival (PFS). Chemotherapy had no impact on PFS in early-stage disease (FIGO I-II). The PFS was similar using BEP or other chemotherapy regimens (HR 0.88 [0.43; 1.81]) in the first-line therapy. In case of recurrence, PFS was statistically prolonged by complete surgery, but perioperative chemotherapy use did not impact PFS.
Chemotherapy use did not impact survival in the first-line or relapse setting in SCST. Only surgery and its quality demonstrated benefit for PFS in ovarian SCST in any lines of treatment.

Sclerosing stromal tumor of the ovary (SSTO) is a rare benign neoplasm of the ovary, accounting for about 6% of all sex cord stromal ovarian tumors. Only 17 cases of SSTO occurring during pregnancy have been reported to date. We report a case of SSTO occurring during pregnancy and review the existing literature. A 32-year-old pregnant woman was found to have a 10-cm solid mass in the right adnexa, and a large volume of ascites fluid was detected by ultrasound examination in the second trimester. The patient underwent abdominal puncture to relieve her symptoms in the second trimester, and a partial right oophorectomy and cesarean section were performed at 39 weeks of gestation. Final pathology confirmed the diagnosis of SSTO. Both the mother and baby were well at 6 months of postpartum follow-up. Clinical symptoms, and hormone and imaging examinations are all helpful in making a differential diagnosis of SSTO, but the unique histopathological and immunohistochemistry findings remain the main diagnostic features. Pregnant women with SSTO usually undergo enucleation or unilateral oophorectomy, which generally does not have adverse pregnancy outcomes.

This article reviews the recent advances and potential future changes in the classification of testicular germ cell and sex cord stromal tumors, highlighting changes in the classification system and terminology with description on newer entities. A discussion on approaching difficult areas and diagnostic pitfalls is also included along with the utility of ancillary investigations. Areas with limited knowledge are highlighted to providing direction for future studies and a bulleted summary in the form of critical care points is provided.

Background: DICER1 tumor predisposition syndrome is characterized by an increased risk for development of pleuropulmonary blastoma, pituitary blastoma, multinodular thyroid goiter, thyroid carcinoma, sex cord stromal tumor, cystic nephroma, embryonal rhabdomyosarcoma, and tumors of the CNS, amongst others. Of this list, only pituitary blastoma is recognized as pathognomonic for the syndrome. Case report: We describe a 15-year-old female with bilateral, asynchronous Sertoli-Leydig cell tumors (SLCT). Both tumors harbored an identical germline frameshift mutation as well as unique somatic DICER1 hot-spot point mutations. Discussion: A review of bilateral SLCTs demonstrates that all patients with available DICER1 mutation status carried a germline DICER1 mutation (100%, 9 of 9). In cases with known somatic DICER1 status on bilateral tumors, all harbored distinct somatic mutations (100%, 5 of 5). Our findings support the notion that bilateral ovarian SLCTs are indeed separate events and do not represent recurrent or metastatic disease.

Adult type granulosa cell tumours are extremely rare. Albeit mostly benign, 10% have malignant potential associated with unfavorable pathological features. The present case with the longest history duration, shows that size alone may not necessarily be unfavorable prognosticator when not associated with other factors.