Synchronous uterine serous carcinoma and ovarian sex cord stromal tumor (thecoma)–A rare first case report

Synchronous endometrial and ovarian carcinoma is a rare instance and it accounts for 50 to 70% of all synchronous female genital tract tumors. However, it is very rare to find synchronous endometrial carcinoma and ovarian sex cord–stromal tumor (thecoma). The present case is a 75-year-old woman with a complaint of post-menopausal vaginal bleeding. Radiologically, the magnetic resonance imaging (MRI) pelvis revealed altered signal intensity mass in the uterus. Frozen section and routine histopathological examination were done on radical hysterectomy. Microscopically, serous carcinoma involving uterine corpus and left Fallopian tube was identified along with the unusual finding of contralateral ovarian sex cord–stromal tumor (thecoma), which was confirmed on immunohistochemical examination. It is a very rare association and is first reported in the present study after a thorough search of the published literature. Their relationship based on a high level of estrogen produced by the hyperactive ovary is controversial as serous carcinomas are less hormone-dependent.

Tumor de células de Sertoli-Leydig: a propósito de un caso y revisión de la literatura reciente / Ovarian Sertoli-Leydig cell tumour: a clinical case and recent basic literature review

INTRODUCCIÓN: Los tumores de células de Sertoli-Leydig son neoplasias de ovario infrecuentes, lo que dificulta su diagnóstico y tratamiento. OBJETIVO: Revisar y sintetizar el manejo actual de los tumores de células de Sertoli-Leydig. MÉTODO: Se realizó una revisión de la literatura reciente sobre tumores de células de Sertoli-Leydig, a propósito de un caso en nuestro centro. RESULTADOS: Los tumores de las células de Sertoli-Leydig son infrecuentes, con mayor incidencia en edades tempranas. Ante una paciente joven con una lesión anexial unilateral y signos de virilización deberán considerarse estos tumores dentro del diagnóstico diferencial. En los estadios iniciales y en pacientes jóvenes podrá plantearse un tratamiento quirúrgico que preserve la fertilidad, y la asociación de tratamiento adyuvante dependerá de la diferenciación y del estadiaje del tumor.

INTRODUCTION: Sertoli-Leydig cell tumors are infrequent ovarian neoplasms, which difficults their diagnosis and treatment. Objective: To review and synthesize the current management of the Sertoli-Leydig cell tumor. METHOD: A review of the recent literature regarding the Sertoli-Leydig cell tumor was carried out, regarding a case in our center. RESULTS: Sertoli-Leydig cell tumors are an infrequent entity, with a higher incidence in early ages. In a young patient with a unilateral adnexal lesion and signs of virilization, these tumors should be considered within the differential diagnosis. In early stages and young patients, a surgical treatment that preserves fertility may be considered, and the association of adjuvant treatment will depend on the differentiation and staging of the tumor.

Preoperative differentiation of benign and malignant non-epithelial ovarian tumors: clinical features and tumor markers / Diferenciação pré-operatória de tumores ovarianos não epiteliais benignos e malignos: características clínicas e marcadores tumorais

Abstract Objective To evaluate the role of clinical features and preoperativemeasurement of cancer antigen 125 (CA125), human epididymis protein(HE4), and carcinoembryonic antigen (CEA) serum levels in women with benign and malignant non-epithelial ovarian tumors. Methods One hundred and nineteen consecutive women with germ cell, sex cordstromal, and ovarian leiomyomas were included in this study. The preoperative levels of biomarkers were measured, and then surgery and histopathological analysis were performed. Information about the treatment and disease recurrence were obtained from the medical files of patients. Results Our sample included 71 women with germ cell tumors (64 benign and 7 malignant), 46 with sex cord-stromal tumors (32 benign and 14 malignant), and 2 with ovarian leiomyomas. Among benign germ cell tumors, 63 were mature teratomas, and, amongmalignant, fourwere immatureteratomas. Themost common tumors in the sex cordstromal group were fibromas (benign) and granulosa cell tumor (malignant). The biomarker serum levels were not different among benign andmalignant non-epithelial ovarian tumors. Fertility-sparing surgeries were performed in 5 (71.4%) women with malignant germ cell tumor. Eleven (78.6%) patients with malignant sex cord-stromal tumors were treated with fertility-sparing surgeries. Five women (71.4%) with germ cell tumors and only 1 (7.1%) with sex cord-stromal tumor were treated with chemotherapy. One woman with germ cell tumor recurred and died of the disease and one woman with sex cord-stromal tumor recurred. Conclusion Non-epithelial ovarian tumors were benign in the majority of cases, and the malignant caseswere diagnosed at initial stages with good prognosis. Themeasurements of CA125, HE4, and CEA serum levels were not useful in the preoperative diagnosis of these tumors.

Resumo Objetivo Avaliar o papel das características clínicas e a medida pré-operatória dos níveis séricos de CA125, HE4, e CEA em mulheres com tumores de ovário não epiteliais benignos e malignos. Métodos Cento e dezenovemulheres consecutivas comtumores ovarianos de células germinativas, do cordão sexual-estroma, e miomas ovarianos foram incluídas neste estudo. Os níveis pré-operatórios dos biomarcadores foram medidos, a cirurgia e a análise histopatológica foram realizadas. Informações sobre tratamento e recorrência da doença foram obtidas dos prontuários médicos das pacientes. Resultados Nossa amostra incluiu 71 mulheres com tumores de células germinativas (64 benignos e 7 malignos), 46 com tumores do cordão sexual-estroma (32 benignos e 14 malignos), e 2 com leiomiomas ovarianos. Entre os tumores benignos de células germinativas, 63 eram teratomas maduros, e, entre os malignos, quatro eram teratomas imaturos. Os tumores mais comuns do grupo do cordão sexual-estroma foram fibromas (benignos) e tumores de células da granulosa (malignos). Os níveis séricos dos biomarcadores não diferiram entre os tumores de ovário não epiteliais benignos e malignos. A cirurgia preservadora de fertilidade foi realizada em 5 (71,4%) mulheres com tumores malignos de células germinativas. Onze (78,6%) mulheres com tumores do cordão sexual-estromamalignos foram tratadas comcirurgia preservadora de fertilidade. Cinco (71,4%)mulheres com células germinativas e apenas 1 (7,1%) com tumor do cordão sexual-estroma foram tratadas com quimioterapia. Uma mulher com tumor de células germinativas recidivou e morreu da doença. Uma mulher com tumor do cordão sexual-estroma recidivou. Conclusão Os tumores de ovário não epiteliais foram benignos namaioria dos casos e os malignos foram diagnosticados em estágios iniciais, com bom prognóstico. A medida dos níveis séricos de CA125, HE4, e CEA não foram úteis no diagnóstico préoperatório desses tumores.

Unusual metastatic sex cord stromal tumor - staging and response assessment

Metastatic sex cord stromal tumor (SCST) is rare and contributing <1% of ovarian tumors. A 42-year-old female presented with lower abdominal pain for 2 months. Ultrasonography revealed masses in the abdominal and pelvic. USG guided biopsy from the pelvic mass revealed SCST. For staging, whole body F-18 fluorodeoxyglucose positron emission tomography-computed tomography was performed and revealed heterogenous mildly FDG avid retroperitoneal and bilateral pelvic masses with lung nodules. To confirm the nature of the lung nodule; PET-CT guided biopsy performed and revealed metastatic SCST. After three cycle of chemotherapy, clinical, biochemical and imaging show partial response to treatment.

Ovarian fibrothecoma with minor sex cord elements: a case report

Ovarian fibroma is the most common sex cord stromal tumour of ovary accounting to 1-5% of all ovarian tumours. Minor sex cord elements in ovarian fibroma are a rare entity occupying less than 10% of tumour area. To the best of our knowledge only 20 cases has been reported till date. This case is presented because of its rarity. Authors reported a case of fibrothecoma with minor sex cord elements in a 70yr old postmenopausal women who presented with postmenopausal bleeding with abdominal mass.

Uterine tumor resembling ovarian sex cord tumor: A series of six cases displaying varied histopathological patterns and clinical profiles

Introduction: Uterine tumors resembling ovarian sex cord tumor (UTROSCT) are a unique group of neoplasms with diverse morphology and immunophenotypic characteristics, coexpressing sex cord, epithelial, and smooth-muscle markers. To date, less than 100 cases have been reported and there is paucity of data concerning their clinical behavior. Materials and Methods: All cases of uterine body tumors diagnosed over a period of two and a half years (2016-2018) were retrieved. Histopathological features were reviewed and extended panel of immunohistochemistry was performed to identify cases of UTROSCTs. Results: Six cases of UTROSCTs were identified with a median age of 46.5 years. Four of them presented with menorrhagia, while two with postmenopausal bleeding including one with a history of carcinoma breast. Three of these cases were initially misdiagnosed as endometrial stromal sarcoma and adenocarcinomas. They all underwent hysterectomy with bilateral salpingo-oophorectomy. Conclusion: It is considered a tumor with low malignant potential; however, one out of six cases (16.7%) in our study showed metastasis, within 1 year of diagnosis. It is important to recognize this entity as it mimics a wide range of both benign and malignant tumors. Molecular pathogenesis and exact management protocols remain elusive due to rarity,hence, multi-institutional studies are warranted.

A rare case of steroid cell tumor of ovary: case report

Steroid cell tumors of the ovary account for less than 0.1% of all ovarian tumors. These tumors may present at any age with presentations related to the hormonal activity and virilizing properties of tumor. A 61-year-old postmenopausal women presented with complaints of postmenopausal bleeding for 15 days. Parity score of P3L3, not tubectomised. Menopaused 16 years back. History of weight loss noted. She is a known case of diabetes mellitus for 6 years not on any treatment and a known case of depressive disorder for 35 years on treatment on trihexphenidyl lurasidone. Had undergone sigmoid colon polyp removal in June 2018. On examination, P/A- mild gaseous distension (+). P/S- cervix flushed with vagina, pulled up cervix. P/V- uterus size and position couldn’t be made out, anterior fornix fullness (+). Ultrasonography showed bulky uterus with fibroid 4x4cm, endometrial thickness- 9 mm? Krukenberg tumor and posterior mediastinal lymph nodes. Patient underwent Total abdominal hysterectomy with bilateral salpingo-oophorectomy with frozen section on 01/07/19. Frozen section: 1. Ovaries: right ovary - fibrothecoma, left ovary- simple cyst. 2. Uterus- endometrial hyperplasia with atypia. 3. Myometrium- leiomyoma and adenomyosis. Postoperative period was uneventful. On microscopic examination, impression: right ovary- steroid cell tumor, uterus-endometrial hyperplasia with cytological atypia. Ovarian steroid cell tumors are grouped under sex chord stromal tumors and are usually benign, unilateral and characterized by a steroid cell proliferation. Steroid cell tumors are associated with androgenic changes with variable frequency, ranging from 12% to over 50% respectively. The primary treatment is surgical extirpation of the primary lesion and there are no reports of effective radiation or chemotherapy. In a young patient with stage IA disease, a unilateral salpingo oophorectomy is adequate.

Sporadic ovarian sex cord-stromal tumor with annular tubules: a rare case report

Ovarian sex cord stromal tumor with annular tubules (SCTAT) is a distinctive, rare subtype of sex cord stromal tumor of the ovary, predominant component of which has morphological features intermediate between that of granulosa cell and sertoli cell. The majority of ovarian SCTAT are benign. So far, malignant behavior in SCTAT has been reported only in sporadic cases. We have presented a case of SCTAT in a 40 year old lady with no association of Peutz-Jegher (P-J) syndrome. The patient’s chief complaints were post-menopausal bleeding for 1 year on and off along with menorrhagia. MRI abdomen was suggestive of intensely enhancing solid tissue mass lesion in the right  adnexa, features suggestive of ovarian mass. Panhysterectomy was done. Grossly uterus and left adnexa appeared to be normal. Right ovary showed mass measuring 17x11x9cm3 in size, on cut section, solid, homogenous lobulated, yellowish areas identified. Microscopic and Immunohistochemistry findings confirmed the diagnosis of sex cord stromal tumor with annular tubules of granulosa cell type. PAS stain supported the diagnosis.

Clinicopathological analysis of ovarian tumors: a two year retrospective study

Background: Ovarian tumours are a heterogeneous neoplasm with a varied clinical, morphological and histological feature. Increasing mortality rate due to ovarian cancers has been reported in recent years. Ovarian tumours in post-menopausal females have high risk of malignancy and it has a very poor outcome. The aim and objective of this study was to determine clinical and histopathological spectrum and the frequency and age distribution of various ovarian tumors.Methods: It is a retrospective observational study of patients with ovarian tumors in the department of obstetrics and gynecology, SGRRI of Medical Health & Sciences and Hospital from January 2016 to December 2017 in a total number of 86 patients. All specimens were sent to pathology department and categorised according to WHO  hispathological classification.Results: Out of 86 cases examined, 64 cases were benign (74.4%), 3 cases were borderline (3.4%) and 19 cases were malignant (22.2%). Majority of the ovarian tumors (73.4%) were seen in the age group of 20 to 50 years. Most commonly encountered benign ovarian tumour was serous cystadenoma (58.1%). Surface epithelial tumors were the commonest tumors (64%) followed by germ cell tumors (29%).Conclusions: A variety of benign and malignant tumours of ovary were reported in this study. Early diagnosis and appropriate treatment of ovarian neoplasms favour the good prognosis. Most common benign tumour encountered in this study was serous cystadenoma.

Malignant Sertoli cell tumor of the testis masquerading as seminoma with bone metastasis

Sex cord–stromal tumors of the testes are rare malignancies as compared to germ cell tumors. Pure Sertoli cell tumors are still rare representing <1% of testicular cancers and the malignant forms are too rare. Furthermore, the occurrence of metastasis in such cases is extremely rare with <30 cases reported in literature so far to the best of our knowledge. We present herein a case of malignant Sertoli cell tumor in a 48-year-old male who was initially misdiagnosed as seminoma based on histology and clinical presentation. Four months later, he presented with symptoms due to bony metastasis and found to have widespread metastatic disease which is a very rare presentation of Sertoli cell tumors. Diagnosis of sex cord–stromal tumor requires high index of suspicion as these tumors are most of the times misdiagnosed as germ cell tumors due to their rare incidence and atypical presentation leading to mismanagement. Timely diagnosis at an early stage can provide therapeutic benefit due to lack of well-defined treatment options at advanced stages.

The imaging diagnosis of the sex cord-stromal tumors of ovary / 实用放射学杂志

Objective To discuss the imaging findings of the sex cord-stromal tumors of ovary.Methods The pathologically confirmed CT and MRI images of 69 patients with sex cord-stromal tumors of ovary were collected retrospectively.Results Among 69 cases of ovarian sex cord-stromal tumors,there were fibrothecoma 3 1 cases,thecal cell tumor 1 9 cases,fibroma 1 2 cases,granulosa cell tumors 5 cases and stromal sarcomas 2 cases.CT and MRI had detected all the 69 tumors.Most of the tumors (52 cases,accounting for 75%)were presented as mainly solid tumors combined with cystic changes,some (13 cases,accounting for 19%)were presented as all solid and a few cases (4 cases,accounting for 6%)were presented as cystic tumors.The tumors were round or oval,with clear boundary,which showed low density,and either no obvious enhancement or mild delayed enhancement on CT scan.The solid part of tumors showed low signal on T2WI and slightly higher signal on diffusion weighted imaging(DWI).Conclusion The sex cord-stromal tumors of ovary are mainly solid tumors combined with cystic changes,with clear boundary,either with no obvious enhancement or mild delayed enhancement and low signal on T2WI and slightly higher signal on DWI.The study shows that the above imaging features are helpful to improve the diagnostic accuracy of sex cord-stromal tumors of ovary.

Possibility of women treated with fertility-sparing surgery for non-epithelial ovarian tumors to safely and successfully become pregnant-a Chinese retrospective cohort study among 148 cases / 医学前沿

This study was performed to evaluate the oncological and reproductive outcomes of childbearing-age women treated with fertility-sparing surgery (FSS) for non-epithelial ovarian tumors in China. One hundred and forty eight non-epithelial ovarian tumor women treated with FSS between January 1, 2000 and August 31, 2015 from two medical centers in China were identified. Progression-free survival (PFS) was 88.5%, whereas overall survival (OS) was 93.9%. Univariate analysis suggested that delivery after treatment is related to PFS (P = 0.023), whereas histology significantly influenced OS. Cox regression analysis suggested that only histology was associated with PFS and OS (P < 0.05). Among the 129 women who completed adjuvant chemotherapy (ACT), none developed amenorrhea. Among the 44 women who desired pregnancy, 35 (79.5%) successfully had 51 gestations including 35 live births without birth defects. Non-epithelial ovarian tumors can achieve fulfilling prognosis after FSS and chemotherapy. Histology might be the only independent prognostic factor for PFS and OS. FSS followed by ACT appeared to have little or no effect on fertility. Meanwhile, postoperative pregnancy did not increase the PFS or OS. Use of gonadotropin-releasing hormone agonist was not beneficial for fertility.

Application of MRI combined with CT on diagnosing ovarian sex cord stromal tumor / 实用放射学杂志

Objective To assess the value of MRI combined with CT in the diagnosis of ovarian sex cord stromal tumor (OSCST).Methods The CT and MRI features of 29 cases with OSCST confirmed by pathology were analyzed retrospectively.Results Fibrothecoma in 19 cases showed a solitary round mass with a regular border,solid in 12 cases (63.1％) and cystic-solid in 7 cases (36.9％),calcification in 2 cases,isointense on T1WI and hypointense or slight hyperintense on T2WI with slight enhancement.Granule cell tumor in 9 cases showed a solitary cystic solid mass with thick wall,without nodules on cystic wall,and slight enhancement in the solid parts.Sertoli-leydig cell tumor in 1 case showed a solid mass,slightly hyperintense on T1WI,hyperintense on T2 WI,and obvious enhancement.Conclusion CT and MRI manifestations of OSCST demonstrate some characteristics.Combination with MRI and CT can improve the accuracy of the diagnosis.

Granulosa cell tumor of testis: Clinicopathological correlation of a rare tumor.

Background: Granulosa cell tumor of testis is a rare tumor accounting for less than 4% of adult testicular tumors though they account for nearly 30% of childhood testicular tumors. Due to the rarity of these tumors, exact etiology, pathogenesis, prognostic factors and best treatment approach are not well known. The molecular events in pathogenesis of these stromal tumors have begun to unravel and these developments put forth a reasonable and scientifi c explanation for the association of these tumors with developmental anomalies like undescended testis. However, many questions remain unanswered. Materials and Methods: We performed a retrospective analysis of clinicopathological features of all Granulosa Cell Tumors of testis from our archives in addition to an extensive literature search using PUBMED with the key words “Granulosa Cell Tumor, testis”. Results: We found six cases in our archives, two of which were of juvenile type and four of adult type. One out of these six cases presented with metastases. All cases underwent radical orchidectomy. Morphology and immunohistochemistry were classical in all cases and there was no diagnostic dilemma. Literature search revealed 63 cases of testicular Granulosa Cell Tumor in addition to highlighting the similarities in the biology and the dissimilarities in the clinical behavior as compared to ovarian Granulosa Cell Tumor. Conclusion: Testicular Granulosa Cell Tumor is a rare tumor, which although histologically similar to its ovarian counterpart, differs in clinical behavior. Further detailed investigations are needed to reveal the mystery behind the differing clinical behavior despite histological and immunohistochemical similarity between the testicular and ovarian Granulosa Cell Tumors.

Alfa-fetoprotein secreting ovarian sex cord-stromal tumor.

Ovarian sex cord-stromal tumors are relatively infrequent neoplasms that account for approximately 8% of all primary ovarian tumors. They are a heterogeneous group of neoplasms composed of cells derived from gonadal sex cords (granulosa and Sertoli cells), specialized gonadal stroma (theca and Leydig cells), and fibroblasts. They may show androgenic or estrogenic manifestations. We report such a tumor associated with markedly raised serum alpha-fetoprotein (AFP) levels in a young female presenting with a mass and defeminising symptoms. Serum AFP levels returned to normal on removal of tumor.

Successful term pregnancies after laparoscopic excision of poorly differentiated Sertoli-Leydig cell tumor of the ovary / 부인종양

Ovarian Sertoli-Leydig cell tumors are rare sex cord-stromal tumors, accounting for less than 1% of ovarian tumors. Majority of these tumors are benign and unilateral, only 3-5% are bilateral. These patients present with clinical features of virilization due to excessive secretion of testosterone from the tumor, however 50% may have no endocrine symptoms. We report a case of poorly differentiated Sertoli-Leydig cell tumour in a woman diagnosed during routine investigation of infertility. She had two spontaneous successful pregnancies after tumor excision laparoscopically.

Sclerosing stromal tumors of the ovary occurred in various ages / 대한산부인과학회지

Sclerosing stromal tumor (SST) of the ovary is a rare, benign tumor. The most common clinical symptom is menstrual irregularity. Diagnosis of SST is often made by postoperative pathologic examination. The important differential diagnoses are other sex cord stromal tumors including fibroma, thecoma and etc. We present four cases of SST of the ovary during 10 years with a brief review of the literature.

Ovarian sex cord-stromal tumours: “newly recognised entities”.

A sex cord-stromal tumour is one that is composed of granulosa cells, theca cells, Sertoli cells, Leydig cells and fibroblasts of stromal origin, singly or in various combinations. A pseudopapillary pattern has been described recently in granulosa cell tumours. This was seen in both juvenile and adult types. Pseudopapillae develop as a secondary or degenerative phenomenon lacking true stromal cores. The distinction of a granulosa cell tumour from a surface epithelial carcinoma can be aided by the use of Epithelial Membrane Antigen (EMA) which is positive in carcinomas and Inhibin and Calretinin is positive in granulosa cell tumours. A negative inhibin on immunohistochemistry does not exclude a diagnosis of granulosa cell tumour.The study by Irving et al (2006) emphasizes the distinction of a group of mitotically active, cytologically bland, cellular fibromatous tumours from fibrosarcomas. These tumours show a mean of 6.7 Mitotic figure (MF)/10 HPF with a range of 4 to !9 MF/ I 0 HPF. Luteinized thecomas occur in younger women than typical the comas, the mean age being 46 years. A distinct variant of luteinized the coma is one that is associated with sclerosing peritonitis. Microcystic stromal tumour is a newly recognized entity. Twelve types of this non-functioning, neoplasm have been described, all occurring in adults. Sertoli cell tumours are rare in their pure form. They often present with oestrogenic manifestations in young women. Occasional cases are seen in the Peutz-Jeghers syndrome.

A case of successful laparoscopic surgical staging in patient of Sertoli-Leydig cell tumor / 대한산부인과학회지

Sertoli-Leydig cell tumors are rare sex cord-stromal tumors. These tumors account for less than 0.5% of all ovarian tumors. Because these tumors appear predominantly in young women (between the age of 30 and 40) and are bilateral in less than 1% of cases, conservative removal of the tumor and adjacent fallopian tube is justifiable. Recently, we experienced a case of successful laparoscopic surgical staging in patient of Sertoli-Leydig cell tumor which was previously incompletely evaluated in local OBGY clinic. There are no solid data to suggest that usefulness and risk of laparoscopic surgical staging and adjuvant therapy with stage I disease of these tumors until now. So we present it with a brief review of literature.

A case report of ovarian sex cord-stromal tumor, unclassified type / 부인종양

Unclassified type occupies about 5 to 10 percent of all ovarian sex cord stromal tumors. Diagnosis is very difficult and subjective because tumors show insufficient differentiation to ovarian or testicular line or display insufficient findings to diagnose as a gynandroblastoma in spite of divergent differentiation. To our knowledge sixty-two cases have been reported in the pathology literatures as yet. The behavior of this group of tumors has not been adequately studied but is similar to granulose cell tumors or Sertoli-Leydig cell tumors. We present a case of unclassified sex cord-stromal tumor which has been experienced in our hospital with brief review of the literature.