ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
ABSTRACT
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.
Subject(s)
Female , Humans , Pregnancy , Accounting , Infertility , Laparoscopy , Ovary , Sertoli-Leydig Cell Tumor , Sex Cord-Gonadal Stromal Tumors , Testosterone , VirilismABSTRACT
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.
Subject(s)
Female , Diagnosis, Differential , Fibroma , Ovary , Sex Cord-Gonadal Stromal Tumors , ThecomaABSTRACT
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.
Subject(s)
Female , Humans , Fallopian Tubes , Sertoli-Leydig Cell Tumor , Sex Cord-Gonadal Stromal TumorsABSTRACT
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.
Subject(s)
Female , Diagnosis , Ovary , Pathology , Sertoli-Leydig Cell Tumor , Sex Cord-Gonadal Stromal TumorsABSTRACT
Sex cord stromal tumors (GCT) of the ovary compose just 5% of ovarian tumors. Most of them are granulosa cell tumors (GCT). There are two types of tumors, juvenile (JGCT) and adult type (AGCT), which have different clinical and histopathological features. JGCT represents only 5% of GCT. GCT is characterized by secretion of estrogen. Patients may present with vaginal bleeding in adult type, and sexual pseudoprecocity in juvenile type, as results of prolonged exposure to tumor-derived estrogen. Surgery is a principle of treatment and required for definite tissue diagnosis, staging, and tumor debulking. Survival of patients with GCT is generally excellent because most patients present with early stage disease. Because of the propensity of GCT to recur years after initial diagnosis, prolonged surveillance such as physical examination and serum tumor markers such as estradiol and inhibin is reasonable. We present 5 cases GCT, 4 AGCT and 1 JGCT, with brief review of literature.
Subject(s)
Adult , Female , Humans , Diagnosis , Estradiol , Estrogens , Granulosa Cell Tumor , Granulosa Cells , Inhibins , Ovary , Physical Examination , Sex Cord-Gonadal Stromal Tumors , Biomarkers, Tumor , Uterine HemorrhageABSTRACT
Juvenile granulosa cell tumor is a well-known sex-cord stromal ovarian neoplasm different in appearance and behavior from the adult granulosa cell tumor and occurs most (80%) frequently in the first two decades. The prognosis of juvenile granulosa cell tumor in children is good in most cases, but correlated with stage, size, the presence of ruptures, the grade of nuclear atypia, the degree of mitotic activity. The surgery remains the principal line of treatment for low stage juvenile granulosa cell tumor in children, but for those patient with high stage juvenile granulosa cell tumor or recurrence, the best treatment has yet to be determined and tumor sensitivity to radiation therapy and chemotherapy is not clearly. We present a case of juvenile granulosa cell tumor with brief review of literatures.
Subject(s)
Adult , Child , Female , Humans , Drug Therapy , Granulosa Cell Tumor , Granulosa Cells , Ovarian Neoplasms , Ovary , Prognosis , Recurrence , RuptureABSTRACT
We described a case of mixed germ cell-sex cord-stromal tumor of non-gonadoblastoma type (GCSCT-NG) of the ovary in a 2-year-old girl and compared its histological, immunohistochemical and ultrastructural findings with those of gonadoblastoma. The germ cell component showed an immunopositivity for cytokeratin and epithelial membrane antigen (EMA), and a negative reaction for -inhibin, alpha-fetoprotein (AFP), placental alkaline phosphatase (PLAP), vimentin and CD99. The sex cord elements showed an immunopositivity for cytokeratin, EMA, vimentin and inhibin, but negativity for AFP, PLAP, and CD99. The ultrastructural features showed desmosome-like cell junctions, suggesting spermatocytic differentiation from the primordial germ cells or gonocytes. Immunohistochemical and ultrastructural findings suggested that GCSCT-NG is probably a more differentiated form of mixed germ cell- sex cord-stromal tumor than gonadoblastoma.
Subject(s)
Child, Preschool , Female , Humans , Alkaline Phosphatase , alpha-Fetoproteins , Germ Cells , Gonadoblastoma , Inhibins , Intercellular Junctions , Keratins , Mucin-1 , Ovary , Sex Cord-Gonadal Stromal Tumors , VimentinABSTRACT
Mucinous neoplasms occur rarely in association with cystic teratoma, Sertoli-Leydig cell tumor, granulosa cell tumor or carcinoid tumor. Several cases of an ovarian stromal tumor with minor sex-cord elements have been reported in the literatures. However, there has been no report about an ovarian mucinous neoplasm coexisting with a stromal tumor with sex-cord elements yet. We report a case of an ovarian neoplasm composed of both mucinous cystadenoma and stromal tumor with minor sex-cord elements in a 58-yr-old female. The ovary including the mass measured 5 cm in size. On section, it revealed an unilocular cyst (4.5 cm in diameter) filled with mucinous fluid. There was a round, yellow, solid nodule, 1.5 cm in diameter within the wall. Microscopically, the cyst was lined by a single layer of endocervical mucinous epithelium and the nodule was composed of spindle cells showing an intersecting and whorled arrangement. There were cell nests showing polygonal shape with abundant cytoplasm among the spindle cells. They showed immunoreactivity for inhibin and did not have any connection with the adjacent mucinous epithelium. Therefore, we interpret the mucinous cystadenoma as having arisen de novo.
Subject(s)
Female , Humans , Cystadenoma, Mucinous/chemistry , Inhibins/analysis , Middle Aged , Ovarian Neoplasms/chemistry , Sertoli-Leydig Cell Tumor/pathology , Stromal Cells/pathologyABSTRACT
Sex cord-stromal tumors of the ovary are the third most common types of neoplasms that develop in the ovary and account for about 5-8% of all ovarian malignancies. This group of ovarian neoplasms is derived from the sex cords and the ovarian stroma or mesenchyme. Granulosa-stromal cell tumors include granulosa cell tumor, thecoma and fibroma. The granulosa cell tumor is a low-grade malignancy and accounts for about 2-3% of all ovarian malignancies. Granulosa cell tumors have a prolonged natural history and tendency toward late relapse, reflecting their low grade biology. We present a case of juvenile granulosa cell tumor of the ovary with brief review of literature,
Subject(s)
Female , Biology , Fibroma , Granulosa Cell Tumor , Granulosa Cells , Mesoderm , Natural History , Ovarian Neoplasms , Ovary , Recurrence , Sex Cord-Gonadal Stromal Tumors , ThecomaABSTRACT
The authors observed a case of malignant mixed germ cell-sex cord stromal tumor occuring in a 43-year-old woman. A laparotomy perfomed on August 3, 1994, revealed a large roughly round, partially ruptured tumor in the right ovary. The tumor mass weighed 330 gm and its dimensions were 11X9X8 cm. Tumors composed of germ cells and sex cord stroma intimately associated togther are very rare. Interest in these gonadal tumors that are distinct from gonadoblastoma has mouted in recent years. The tumor behaves in a benign clinical course and occurs predominantly in female infats and children with normal 46.XX karyoytype. This tumor differs from gonadobalstoma in its histologic pattern, absence of regresive changes, lack of association with other neoplastic germ cell elements, and occurrence in normal gonads.Histologically, the mass was characterized by an intimate admixutr of germ cells and sex cord stromal cells. The lesion had many predominant setoli tuble formations and lipid cell changes. But it lacked Call-Exner like bodies, calcification and byalinization that charaterize gonadoblastomas. Typical areas of dysgemonoma and endodermal sinus tumor were also present the patient subsequelty received chemotherapy(blemycin,etoposied, and cisplatin administered in 5cycles), The patient was symptom free two yares after surgery and chemotherapy.
Subject(s)
Adult , Child , Female , Female , Humans , Cisplatin , Drug Therapy , Endodermal Sinus Tumor , Germ Cells , Gonadoblastoma , Gonads , Laparotomy , Ovary , Stromal CellsABSTRACT
Sex-cord stromal tumors of ovary account for 5~8% of all ovarian malignancies. This group of ovarian neoplasms is derived from the sex cords and the ovarian stroma or mesenchyme. Although they can occur in women of all ages, they tend to have peak incidence in those over age50. So they should be surgically managd with hysterectomy and bilateral salpingo-oophorectomy. The purpose of this study is to report the experience at Asan Medical Center, Department of Obstetrics and Gynecology, in 24 patients with ovarian sex-cord stromal tumortreated between May, 1989 and April, 1995. We analyzed the effect of histologic subtype, FIGO stage, age and management. The results were as follows: 1. In histologic subtypes, fibrothecoma(66.7%), granulosa cell tumor(20.8%), Sertoli-Leydig cell tumor(8.3%), others(4.0%) were counted in order. 2. The mean age of sex cord-stromal tumor was 44.0 years and 8 cases(33.4%) of tumors over the age of 50.0 years, 13 cases(54.2%) over the age of 40.0 years. 3. Main initial symptoms were abdominal pain(37.5%), asymptomatic cases(25.0%), abdominal mass palpation(16.6%), irregular menstruation(12.5%) in order. 4. Management was surgery only(91.7%), surgery and chemotherapy(8.3%). 5. The mean disease free survival was 30.6 months.
Subject(s)
Female , Humans , Disease-Free Survival , Granulosa Cells , Gynecology , Hysterectomy , Incidence , Mesoderm , Obstetrics , Ovarian Neoplasms , Ovary , Sex Cord-Gonadal Stromal TumorsABSTRACT
Sex-cord stromal tumors of ovary account for 5~8% of all ovarian malignancies. This group of ovarian neoplasms is derived from the sex cords and the ovarian stroma or mesenchyme. Although they can occur in women of all ages, they tend to have peak incidence in those over age50. So they should be surgically managd with hysterectomy and bilateral salpingo-oophorectomy. The purpose of this study is to report the experience at Asan Medical Center, Department of Obstetrics and Gynecology, in 24 patients with ovarian sex-cord stromal tumortreated between May, 1989 and April, 1995. We analyzed the effect of histologic subtype, FIGO stage, age and management. The results were as follows: 1. In histologic subtypes, fibrothecoma(66.7%), granulosa cell tumor(20.8%), Sertoli-Leydig cell tumor(8.3%), others(4.0%) were counted in order. 2. The mean age of sex cord-stromal tumor was 44.0 years and 8 cases(33.4%) of tumors over the age of 50.0 years, 13 cases(54.2%) over the age of 40.0 years. 3. Main initial symptoms were abdominal pain(37.5%), asymptomatic cases(25.0%), abdominal mass palpation(16.6%), irregular menstruation(12.5%) in order. 4. Management was surgery only(91.7%), surgery and chemotherapy(8.3%). 5. The mean disease free survival was 30.6 months.