Ovarian sclerosing stromal tumor mimicking malignancy: case series and literature review.

BACKGROUND: Sclerosing stromal tumors (SST) are rare ovarian neoplasms that often appear as solid unilateral tumors of the ovary with no specific clinical or radiological presentation. The definitive treatment is surgical removal. CASE PRESENTATION: Our article presents four cases of female patients with sclerosing stromal ovarian tumor with clinical characteristics mimicking malignant ovarian lesions. Interestingly, two of our cases had elevated levels of inhibin B. All patients were treated with surgery (oophorectomy) and had no disease recurrence. CONCLUSION: Tumors' macroscopic features are usually non-specific and often suggestive of possible malignancy, therefore diagnosis is always based on histopathological report.

Sclerosing stromal tumor of the ovary: Two case reports and literature review / Tumor estromal esclerosante de ovario: reporte de 2 casos clínicos y revisión de la literatura

Introduction: Sclerosing stromal tumors (SSTs) are rare benign ovarian tumors. They represent 6% of sex cord stromal tumors. Its preoperative diagnosis is often a challenge due to its similarity to malignant tumors on ultrasound imaging. We present two cases of SSTs to emphasize the consideration of this type of tumors in the differential diagnosis of solid adnexal masses in young women. A review of the literature on the typical ultrasound features, clinical presentation, and management of SSTs was performed. Main symptoms and/or clinical findings: Pelvic pain was the main symptom in both cases. In the first case, transvaginal ultrasound revealed an unilocular solid adnexal mass of 59mm×44mm×45mm with cystic areas and marked peripheral and central vascularization. MRI (magnetic resonance imaging) revealed a 50mm×50mm heterogeneous adnexal mass with a solid peripheral component and a cystic-necrotic center. In the second case, pelvic ultrasound showed a solid cystic adnexal mass of 103mm×77mm with marked peripheral vascularity. Main diagnoses: Postoperative anatomopathological diagnosis in both cases was an ovarian SST.Therapeutic interventions and results. Unilateral laparoscopic salpingo-oophorectomy and oophorectomy, respectively, was performed without incidents. There has been no recurrence during follow-up.Conclusion: It is important to consider SSTs in the differential diagnosis of young women with a unilateral solid-cystic adnexal mass with a high degree of peripheral and central vascularization. Laparoscopic approach together with fertility-sparing techniques should be considered the treatment of choice.(AU)

Introducción: Los tumores esclerosantes del estroma (SST) son tumores benignos raros del ovario. Representan un 6% de los tumores del estroma de los cordones sexuales. Su diagnóstico preoperatorio suele ser un desafío por su similitud ecográfica con los tumores malignos. Presentamos 2 casos de SST para enfatizar la consideración de este tipo de tumores en el diagnóstico diferencial de masas anexiales sólidas en mujeres jóvenes. Se realizó una revisión de la literatura sobre las características ecográficas típicas, la presentación clínica y el manejo de los SST. Principales síntomas y/o hallazgos clínicos: El dolor pélvico fue el síntoma principal en ambos casos. En el primer caso, la ecografía transvaginal reveló una masa anexial unilocular sólida de 59×44×45mm con áreas quísticas y marcada vascularización periférica y central. La resonancia magnética nuclear reveló una masa anexial heterogénea de 50×50mm con componente sólido periférico y un centro quístico-necrótico. En el segundo caso, la ecografía pélvica mostró una masa anexial sólido quística de 103×77mm con marcada vascularización periférica. Diagnósticos principales: El diagnóstico anatomopatológico postoperatorio en ambos casos fue de un SST de ovario.Intervenciones terapéuticas y resultados: Se realizó ooforectomía y salpingooforectomía unilateral laparoscópica, respectivamente, sin incidencias. No se ha producido recidiva durante el seguimiento. Conclusión: Es importante considerar los SST en el diagnóstico diferencial ante mujeres jóvenes con una masa anexial sólido-quística unilateral con un alto grado de vascularización periférica y central. El abordaje laparoscópico junto con técnicas preservadoras de fertilidad deben ser consideradas el tratamiento de elección.(AU)

A rare case of sclerosing stromal tumor of the ovary presenting in pregnancy: a case report and review of the literature.

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.

A Rare Ovarian Tumor: The Sclerosing Stromal You Do Not Expect-A Case Series in the Adolescent Population and a Literature Review.

Sclerosing stromal tumor (SST) is a rare ovarian tumor arising from the sex cord-stromal cells that occurs mainly in young adults during the second and third decades of life and rarely in pediatric and adolescent populations. The objective of this study is to report three illustrative cases of SST in young girls who had undergone surgery at our clinic in or after 2009, and to perform a literature review of this rare ovarian tumor. A retrospective chart review of female patients aged <18 years with a diagnosis of SST treated in a tertiary pediatric hospital was performed. Furthermore, a 10-year review of the SST literature was completed. Three cases of SST at our institution were outlined. After reviewing the literature, 18 SST cases were identified. The mean age at diagnosis was 13.4 years, and the reported clinical presentations were abdominal or pelvic pain and menstrual irregularity. Seven patients had abnormal hormone tests or CA-125 levels. In approximately 30% of cases, conservative surgery was performed, preserving residual ovarian tissue. In conclusion, some preoperative findings may help in suggesting the presence of SST. However, definitive diagnosis can only be made by histopathological examination. It is important to consider this tumor because, given its benign behavior, a conservative approach is preferred, particularly in this age group.

Ovarian collision tumor consisting of sclerosing stromal tumor and mature cystic teratoma complicated with Meigs syndrome: A case report.

Ovarian collision tumors are uncommon and reports of their radiological appearance are even less frequent. The present study reported the world's first case of an ovarian collision tumor consisting of an ovarian sclerosing stromal tumor and a mature cystic teratoma and its imaging presentation. When a cystic solid ovarian mass combined with ascites and elevated CA125 is encountered it is frequently diagnosed as a malignant tumor, but the present case was a benign tumor. Therefore, when encountering similar cases, clinicians should not limit the diagnosis to malignant tumors to avoid rashly expanding the surgery and causing unnecessary harm to the patient. The combination of computed tomography, magnetic resonance imaging and pathology findings presented in the current study enable radiologists to learn about this disease and further assist clinicians in developing the best treatment plan.

Ultrasonographic and pathological features of sclerosing stromal tumor: a case report.

Background: We herein report a rare case of a sclerosing stromal tumor (SST) in an adolescent. In this case, the mass displayed a shrinking trend, combined with its ultrasonic manifestations and pathological characteristics and may provide some references for the selection and timing of treatment, to avoid excessive harm to patients. Case Description: A healthy 17-year-old adolescent female presented to the outpatient department, complaining of abnormal uterine bleeding, but no abdominal pain, bloating, chills, or fever. The patient had no history of malignant tumors, and no relevant family or genetic history. An ultrasound showed an inhomogeneous hypoechoic area (106 mm × 53 mm × 68 mm) in the right ovarian, a clear boundary, an anechoic area inside and blood flow was observed in the mass. At a follow-up regular re-examination, the mass displayed a shrinking trend from 95 mm × 50 mm × 88 mm, 61 mm × 28 mm × 42 mm, 43 mm × 28 mm × 40 mm, 43 mm × 28 mm × 40 mm, to 42 mm × 23 mm × 28 mm. The patient underwent laparoscopic surgery a week later. Based on the immunohistochemistry and morphology results, the posterior ovarian mass was diagnosed as an SST. At one month after operation, there was no obvious abnormality on ultrasound. Conclusions: The incidence of SST is relatively low. However, due to the low specificity of clinical manifestations, imaging examination and serum tumor markers, the diagnosis of SST mainly relies on pathological examination. Therefore, in clinical practice, the possibility of misdiagnosis is greater, and attention should be paid to the differentiation of ovarian malignant tumors. Surgical resection is recommended, and the effect is good. Surgical methods should be selected individually according to the size of the tumor and the age of the patient.

Sclerosing stromal tumor: a rare ovarian neoplasm

Sclerosing stromal tumor (SST) is an extremely rare and distinctive sex cord stromal tumor, which occurs predominantly in the second and third decades of life. SSTs make up 2-6% of ovarian sex-cord stromal tumors. Due to the solid and distinct vascular structure of the tumor, it can be mistaken as a number of malignant ovarian tumors. As this specific neoplasm is very rare, it is not always possible to diagnose the tumor preoperatively with clinical and ultrasonographic findings. Furthermore, histopathological and immunohistochemical analysis does not always confirm the diagnosis. In this case report, clinical findings, histopathological features, and macroscopic appearance during laparoscopy of an SST are presented in a 20-year-old woman with pelvic pain. SST should be considered among the differential diagnosis of women with adnexal masses.

Minimally Invasive Ovarian-Preserving Approach for the Management of a Sclerosing Stromal Tumor in an Adolescent: A Case Report.

BACKGROUND: Sclerosing stromal tumors (SSTs) are rare benign ovarian tumors that occur in adolescents and young adults. They are often treated with unilateral salpingo-oopherectomy due to concern for malignancy. CASE: A 13-year-old postpubertal female presented with sharp, constant abdominal pain with physical exam concerning for a lower abdominal mass. An ultrasound revealed a 9.7-cm solid, heterogenous left ovarian mass. The abdomen and pelvis CT confirmed the findings and showed a predominantly cystic mass arising from the left adnexa. During surgery, a smooth and distinct mass arising from the left ovary was encountered without abnormal findings in surrounding structures. A cystectomy was performed, and intraoperative findings showed no evidence of malignancy. Based on the lab, imaging, and surgical findings, staging and salpingo-oopherectomy were not pursued. SUMMARY AND CONCLUSION: Given the benign nature of SSTs, it is important to entertain the diagnosis in adolescents presenting with clinically congruent ovarian masses. The minimally invasive approach allows for sparing of the ovary in the adolescent population.

Sclerosing stromal tumor of the ovary with masculinization, Meig's syndrome and CA125 elevation in an adolescent girl: A case report.

BACKGROUND: Sclerosing stromal tumor (SST) is an extremely rare sex cord stromal tumor of the ovary. It was first reported and named in 1973. These tumors typically present with pelvic/abdominal pain and tenderness, a mass, and/or abnormal menses, but rarely present with masculinity in children and adolescents. Only 2 cases of these tumors have been reported in premenarchal girls, who demonstrated hormonal activity, with a history of the development of a virilizing female due to hyperandrogenism. Here, we report a case of a giant SST with obvious masculinity combined with Meig's syndrome and CA125 elevation. CASE SUMMARY: A 17-year-old female presented with a 7-year history of the development of masculinity and a 2-year history of amenorrhea. She had hirsutism, acne, obvious laryngeal prominence, and voice deepening. Physical examination showed a male suprapubic hair pattern and a 4.0 cm × 1.5 cm enlarged clitoris. Laboratory tests showed that the testosterone level was > 15.00 ng/mL (normal range: 0.14-0.76 ng/mL), and androstenedione level was > 10.00 ng/mL (normal range: 0.3-3.3 ng/mL). A computed tomography scan of the abdomen and pelvis was carried out and showed a large, solid and cystic, partly calcified pelvic mass in the right ovary measuring 27.1 cm × 20.0 cm × 11.0 cm, 15 cm above the umbilicus (to the level of the upper part of L1). Intraoperative findings at laparotomy revealed a large tumor arising from the right ovary. Approximately, 500 mL of pale-yellow clear liquid was found in the pelvic cavity. A right salpingo-oophorectomy was performed. Microscopic examination and immunohistochemical staining of the surgical specimen showed an SST of the ovary. CONCLUSION: This report is remarkable as our patient was not only diagnosed with an SST of the ovary, which is extremely rare in this age group, but was the largest and most obvious reported patient with this tumor who presented with virilization. Therefore, gynecologists should be aware of this potential complication in adolescent girls with a mass in the ovary.

A 10-Month-Old Infant Presenting With Signs of Precocious Puberty Secondary to a Sclerosing Stromal Tumor of the Ovary in the Absence of Hormonal Elevation.

Precocious puberty in an infant is an alarming and infrequent finding, making the differential diagnosis difficult for practitioners. Precocious puberty secondary to a sclerosing stromal tumor (SST) of the ovary is rare. We present a case of a child that began precocious puberty at 3 months of age including development of breast buds, pubic hair, growth spurt, and menarche 5 days prior to presenting to pediatric endocrinology at 10 months. She underwent right salpingo-oophorectomy which demonstrated a soft tissue mass occupying almost the entire ovary with a tan-pink fleshy cut surface. Histological examination confirmed a variant of SST. This case represents an extremely young onset of precocious puberty secondary to a variant of SST without hormonal elevation.

Diversity of imaging features of ovarian sclerosing stromal tumors on MRI and PET-CT: a case report and literature review.

BACKGROUND: Sclerosing stromal tumors (SST) are rare, benign tumors classified as sex cord stromal tumors. To our knowledge, positron emission tomography with computed tomography (PET-CT) findings of SST have only been described in one report and imaging findings on diffusion-weighted imaging (DWI) have only been described in three reports. Characteristic imaging features of SST on PET-CT and DWI have not yet been identified. Here we report a case of multilocular SST with solid components showing mild FDG uptake and slight hyperintensity on DWI, and reviewed the literature. CASE PRESENTATION: Seventeen-year-old woman presented with a complaint of abdominal pain and was admitted due to infectious colitis. Ultrasonography incidentally revealed a multiseptated cystic mass in the pelvis. Magnetic resonance imaging (MRI) showed a large multilobulated cystic mass with irregularly thickened septa and solid components originating in the left adnexa. On T2WI, the cystic components had the same signal intensity (SI) as water, and the irregularly thickened septa and solid components showed intermediate SI higher than the SI of the uterine myometrium. The septa and solid components also showed early strong enhancement on contrast-enhanced T1WI and slight hyperintensity on DWI. The PET-CT showed mild FDG uptake in the solid components of the tumor (SUV: 2.11). According to previous articles, the morphology of SSTs are various; solid mass, well-circumscribed multilobular mass, well-demarcated mass, and multilocular cysticmass. According to the reports describing DWI findings of SST, the SI varies from significant hyperintensity to slightly hyperintensity like in this case. Only one report describing PET-CT findings of SST showed intense FDG uptake (SUV max: 7.0). CONCLUSION: The findings on DWI and PET-CT of our case and the past reports describing PET and DWI findings of SSTs are not consistent. The wide variety of the signal intensity on MRI and FDG uptake on PET could be due to the pathological diversity caused by the cellular areas undergoing collagenous sclerosis, which transforms the tumor into admixture of the collagen and the densely fibrous components with edema.

Different Presentations of Five Rare Cases of Sclerosing Stromal Tumor of the Ovary.

A rare subtype of preceding neoplasm of ovary is sclerosing stromal cell tumor with few presented cases in the literature. In these case series, we describe five cases of ovarian sclerosing stromal cell tumor with different presentations in our department. Interestingly, one of our cases had elevated alfa-fetoprotein.

Clinicopathological Characteristics of Ovarian Sclerosing Stromal Tumor with an Emphasis on TFE3 Overexpression.

A sclerosing stromal tumor is a very rare benign sex cord-stromal tumor of the ovary. Because its clinical presentation and imaging findings are similar to those of borderline or malignant epithelial tumors and other sex cord-stromal tumors, accurate preoperative clinical diagnosis can be difficult. The aim of this study was to analyze the clinicopathological characteristics of SSTs and examine the immunohistochemical expression TFE3, which has not been studied in SSTs. Our study cohort consisted of 9 patients diagnosed as having SST; the median age was 36 years. Radiologically, SSTs presented as multiseptated cystic masses, mixed echoic masses, pseudolobular masses, solid pelvic masses, or uterine subserosal nodules. In 4 of the 9 cases, the preoperative clinical impression was a borderline or malignant ovarian tumor. SSTs displayed the following histopathological features: 1) relatively well-circumscribed cellular nodules that were randomly distributed in the fibrous or edematous stroma; 2) a characteristic alternating pattern of hypercellular and hypocellular areas; 3) a hemangiopericytoma-like vascular growth pattern in the cellular nodules; 4) bland-looking spindle-shaped cells and round or polygonal cells densely clustered around blood vessels; and 5) red blood cell-containing intracytoplasmic vacuole-like spaces in the tumor cell cytoplasm, possibly indicating epithelioid hemangioendothelioma. Immunohistochemically, the tumor cells exhibited diffuse and moderate-to-strong TFE3 expression in 7 of the 9 SSTs. TFE3 was strongly expressed in the nuclei of round or polygonal cells and lutein cells. In contrast, neither luteinized thecomas nor fibromas appreciably expressed TFE3. In summary, our study describes characteristic histopathological features that may be useful for differentiating SSTs from other sex-cord stromal tumors and demonstrates for the first time that SSTs show strong TFE3 expression. Further investigations are necessary to clarify the role of TFE3 in the development of SSTs.

Sclerosing Stromal Tumor Mimicking a Pregnancy Luteoma: Case Report of a Diagnostically Challenging Entity Further Complicated by the Presence of Metastatic Signet Ring Cell Carcinoma From the Stomach.

Sclerosing stromal tumors of the ovary are very rare. We report the case of a 29-year-old pregnant woman who presented with a history of preterm labor and gestational hypertension. A huge mass measuring 30 cm in greatest dimension was recognized on the right ovary during cesarean section. Signet ring cell infiltration was seen during intraoperative frozen section consultation. She underwent a right salpingo-oophorectomy, and the permanent diagnosis was sclerosing stromal tumor infiltrated by signet ring cells. Pregnancy-related changes were seen in tumor morphology. Signet ring cells were immunoreactive with antibodies MUC5ac, MUC2, and EMA. Thus, stromal cells were immunoreactive with antibodies inhibin and calretinin. Endoscopic biopsy of the stomach showed a signet ring cell carcinoma, from which the metastasis had originated. This is the first reported case in the literature, and is notable for its challenging differential diagnosis, which included a pregnancy luteoma and primary signet ring stromal tumor of the ovary.

Sclerosing Stromal Tumor: A Rare Ovarian Neoplasm.

Ovarian sex cord-stromal tumors are relatively infrequent neoplasms that account for approximately 8% of all primary ovarian neoplasm. Sex cord-stromal tumors of the ovary include granulosa cell tumors, fibrothecomas, Sertoli-Leydigcell tumors, steroid cell tumors, and sclerosing stromal tumors (SST). Sclerosing stromal tumors account for 2% to 6% of ovarian stromal tumors. Despite the rarity of this particular neoplasm, it is not always possible to predict the presence of this tumor preoperatively on the basis of clinical and sonographic findings. Histopathological and immunohistochemical (IHC) examinations confirm the diagnosis. Herein, the clinical findings and histopathological features of SST are described in a 24-year-old female.

CT findings of sclerosing stromal tumor of the ovary: A report of two cases and review of the literature.

Sclerosing stromal tumor (SST) of the ovary, which was first described by Chalvardjian and Scully in 1973, is a rare ovarian neoplasm, occurring predominantly in young women. The most common clinical symptom in patients with SST is menstrual irregularities. Microscopically, the tumor is characterized by the presence of pseudo-lobulated cellular areas, with a prominent tendency to sclerosis, marked vascularity and pronounced variation in cellular size and shape. In the current study, 2 cases of SST of the ovary are presented. These cases were confirmed by imaging, surgical and histological examination. No adjuvant therapy was administered to the patients and the two patients were disease-free with no imaging findings of recurrence or metastasis 24 months following surgery.

A Rare Presentation of Paraovarian Sclerosing Stromal Tumor with High Mitotic Activity.

BACKGROUND: Sclerosing stromal tumor is an extremely rare type of benign ovarian sex cord stromal tumor. CASE: The benign characteristic of this tumor is well known but we present an uncommon case of paraovarian sclerosing stromal tumor with high mitotic activity. RESULTS AND CONCLUSION: Despite this potential malignancy, our patient was treated successfully with enucleation only.

Sclerosing stromal tumor of the ovary treated with 2-incision total laparoscopic cystectomy.

Sclerosing stromal tumor (SST) is an extremely rare benign tumor of the ovary that is derived from the sex cord stroma [1,2] and occurs in young women [3-11]. Preoperative diagnosis of the tumor is difficult as it often mimics a malignant tumor. Oophorectomy is usually performed in many cases of young women [3], and diagnosis of SST is made based on post-operative pathological examination. Laparoscopic surgery is seldom performed in SST cases. Here, we report a case of SST of the ovary in an 18-year-old girl who was diagnosed by preoperative imaging and underwent laparoscopic cystectomy. Accurate preoperative imaging helped to perform minimally invasive surgery for SST.