Sclerosing stromal tumour of the ovary presenting as precocious puberty: a rare neoplasm

Chaurasia, Jai Kumar; Afroz, Nishat; Maheshwari, Veena; Naim, Mohammed

doi:10.1136/bcr-2013-201124

Cited by 7 publications

(5 citation statements)

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“…However, in 1975, Damjanov et al reported the production of steroid hormones by SSTs [ 28 ]. Androgens and, less frequently, estrogens can lead to menstrual disturbances and signs of virilization or precocious puberty, as reported in several studies [ 8 , 12 , 21 , 25 , 26 , 27 , 29 , 30 , 31 , 32 , 33 , 34 ]. Menstrual irregularities are very common in the first two years after menarche; therefore, this symptom may be underestimated in this population or simply attributed to a physiologically abnormal hormonal production.…”

Section: Discussionmentioning

confidence: 92%

“…However, the latter is mostly bilateral, lacks the characteristic pseudolobular pattern of sclerosing stromal tumors, and usually occurs during the sixth and seventh decades of life. Furthermore, Krukenberg’s tumor signet ring-like proteins contain mucin rather than lipids, and show nuclear atypia and mitotic activity [ 19 , 34 ]. Sclerosing stromal tumor cells were mainly positive for vimentin, SMA, and inhibin, strongly suggesting their stromal origin.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2023

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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.

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Section: Discussionmentioning

confidence: 92%

Section: Discussionmentioning

confidence: 99%

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

et al. 2023

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“…This entity occurs preferentially in young patients and adolescents, with a mean age at diagnosis of 29 years; postmenopausal cases, however, have been observed [62]. Although patients present with bleeding, menstrual irregularities [63], or pelvic pain [8], SST is typically not associated with hormonal manifestations [61], despite precocious puberty or association with pregnancy having been reported [64,65]. Rare cases have been reported in association with Meigs syndrome [66,67].…”

Section: Sclerosing Stromal Tumormentioning

confidence: 99%

Relevance of Molecular Pathology for the Diagnosis of Sex Cord–Stromal Tumors of the Ovary: A Narrative Review

Trecourt,

Donzel,

Alsadoun

et al. 2023

Cancers

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Ovarian sex cord–stromal tumors (SCSTs) account for 8% of all primary ovarian neo-plasms. Accurate diagnosis is crucial since each subtype has a specific prognostic and treatment. Apart from fibrosarcomas, stromal tumors are benign while sex cord tumors may recur, sometimes with a significant time to relapse. Although the diagnosis based on morphology is straightforward, in some cases the distinction between stromal tumors and sex cord tumors may be tricky. Indeed, the immunophenotype is usually nonspecific between stromal tumors and sex cord tumors. Therefore, molecular pathology plays an important role in the diagnosis of such entities, with pathognomonic or recurrent alterations, such as FOXL2 variants in adult granulosa cell tumors. In addition, these neoplasms may be associated with genetic syndromes, such as Peutz–Jeghers syndrome for sex cord tumors with annular tubules, and DICER1 syndrome for Sertoli–Leydig cell tumors (SLCTs), for which the pathologist may be in the front line of syndromic suspicion. Molecular pathology of SCST is also relevant for patient prognosis and management. For instance, the DICER1 variant is associated with moderately to poorly differentiated SLCTS and a poorer prognosis. The present review summarizes the histomolecular criteria useful for the diagnosis of SCST, using recent molecular data from the literature.

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“…The characteristics of the included studies (18 'adult' [11,12,[20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35] and 16 'paediatric' studies [36][37][38][39][40][41][42][43][44][45][46][47][48][49][50][51]) are provided in Table 1. The mean age of patients included was 10.8 years in the paediatric and 46.9 years in the adult studies.…”

Section: Characteristics Of Included Studiesmentioning

confidence: 99%

MR imaging in discriminating between benign and malignant paediatric ovarian masses: a systematic review

et al. 2019

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Objectives The use of magnetic resonance (MR) imaging in differentiation between benign and malignant adnexal masses in children and adolescents might be of great value in the diagnostic workup of sonographically indeterminate masses, since preserving fertility is of particular importance in this population. This systematic review evaluates the diagnostic value of MR imaging in children with an ovarian mass. Methods The review was made according to the PRISMA Statement. PubMed and EMBASE were systematically searched for studies on the use of MR imaging in differential diagnosis of ovarian masses in both adult women and children from 2008 to 2018.Results Sixteen paediatric and 18 adult studies were included. In the included studies, MR imaging has shown good diagnostic performance in differentiating between benign and malignant ovarian masses. MR imaging techniques including diffusionweighted imaging (DWI) and dynamic contrast-enhanced (DCE) imaging seem to further improve the diagnostic performance. Conclusion The addition of DWI with apparent diffusion coefficient (ADC) values measured in enhancing components of solid lesions and DCE imaging may further increase the good diagnostic performance of MR imaging in the pre-operative differentiation between benign and malignant ovarian masses by increasing specificity. Prospective age-specific studies are needed to confirm the high diagnostic performance of MR imaging in children and adolescents with a sonographically indeterminate ovarian mass. Key Points • MR imaging, based on several morphological features, is of good diagnostic performance in differentiating between benign and malignant ovarian masses. Sensitivity and specificity varied between 84.8 to 100% and 20.0 to 98.4%, respectively. • MR imaging techniques like diffusion-weighted imaging (DWI) and dynamic contrast-enhanced (DCE) imaging seem to improve the diagnostic performance. • Specific studies in children and adolescents with ovarian masses are required to confirm the suggested increased diagnostic performance of DWI and DCE in this population.

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Sclerosing stromal tumour of the ovary presenting as precocious puberty: a rare neoplasm

Cited by 7 publications

References 10 publications

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

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

Relevance of Molecular Pathology for the Diagnosis of Sex Cord–Stromal Tumors of the Ovary: A Narrative Review

MR imaging in discriminating between benign and malignant paediatric ovarian masses: a systematic review

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