scholarly journals Ovarian sex cord-stromal tumors: an update on clinical features, molecular changes, and management

2021 ◽  
pp. ijgc-2020-002018
Author(s):  
Rehab Al Harbi ◽  
Iain A McNeish ◽  
Mona El-Bahrawy

Sex cord stromal-tumors are rare tumors of the ovary that include numerous tumor subtypes of variable histological features and biological behavior. Surgery is the main therapeutic modality for the management of these tumors, while chemotherapy and hormonal therapy may be used in some patients with progressive and recurrent tumors. Several studies investigated molecular changes in the different tumor types. Understanding molecular changes underlying the development and progression of sex cord-stromal tumors provides valuable information for diagnostic and prognostic biomarkers and potential therapeutic targets for these tumors. In this review, we provide an update on the clinical presentation, molecular changes, and management of sex cord-stromal tumors.

2006 ◽  
Vol 4 (2) ◽  
pp. 0-0
Author(s):  
Mindaugas Plečkaitis ◽  
Ugnius Mickys ◽  
Darius Dasevičius

Mindaugas Plečkaitis1, Ugnius Mickys2, Darius Dasevičius21 Vilniaus greitosios pagalbos universitetinė ligoninė,Šiltnamių g. 29 LT-04130, Vilnius2 Valstybinis patologijos centras,Baublio g. 5, LT-08406 VilniusEl. paštas: [email protected]; [email protected] Tikslas Išanalizuoti GIST atvejus, diagnozuotus Valstybiniame patologijos centre 2003–2004 m., apibendrinti histologinius šių navikų požymius, įvertinti histologinių požymių sąsajas su piktybinės biologinės elgsenos rizikos grupėmis. Medžiaga ir metodai Įvertinti pacientų amžių, pasiskirstymą pagal lytį, navikų lokalizaciją, daugybiškumą, metastazes, recidyvus. Papildomai įvertinti histologinius naviko požymius: histologinį piešinį (mišrus, šeivinis, epitelioidinis), organoidinių struktūrų formavimą, ritmines naviko ląstelių branduolių lygiavimosi (palisading) struktūras, ląstelių perinuklearinę vakuolizaciją, naviko miksoidinę stromą, koaguliacinę nekrozę, įsiskverbimą į savąjį gleivinės dangalą (lamina propria), dydį, mitozių kiekį, imunoprofilį. Remiantis navikų dydžiu ir mitozių kiekiu 50-yje didelio padidinimo regos laukų (pagal C.D.M. Fletcher), GIST suskirstyti į piktybinės biologinės elgsenos rizikos grupes. Rezultatai Ištirta 50 GIST atvejų. Ligonių amžiaus vidurkis 64,38 ± 14,26 metų, mediana – 68 metai. Moterų buvo 36, vyrų – 14. Nustatytos 7 (14%) GIST metastazės. Dauginių GIST diagnozuoti 4 atvejai (8%). Šių navikų recidyvų per tirtą laikotarpį nebuvo. Maždaug pusė pavienių navikų rasta skrandyje (45,7%), rečiau – plonojoje žarnoje (15,2%), rečiausiai – storojoje žarnoje (4,3%). Mišrių ir šeivinių GIST kiekis beveik nesiskyrė (atitinkamai 46,2% ir 43,6%), rečiau pasitaikė epitelioidinių navikų (10,3%). GIST dydis svyravo nuo 0,5 cm iki 25 cm (vidurkis 6,4 ± 5,3 cm; mediana 4,9 cm). Dauguma mūsų imunotipuotų GIST buvo teigiami CD 117 ir CD34 žymenims, o pusė navikų – teigiami a-lygiųjų raumenų aktinui. Didesnė dalis į rizikos grupes mūsų suskirstytų pavienių navikų (41,7%) buvo didelės piktybinės biologinės elgsenos rizikos. Koaguliacinė nekrozė buvo 86,7%, o naviko plitimo į savąjį gleivinės dangalą požymiai – 60% didelės piktybiškumo rizikos pavienių GIST. Ki-67 proliferacinis aktyvumas >10% naviko ląstelių populiacijos buvo būdingas didelės rizikos GIST, o Ki-67 proliferacinis aktyvumas <5% nekoreliavo su GIST piktybinės biologinės elgsenos rizikos grupėmis.. Išvada Tyrimo rezultatai parodė, kad naviko koaguliacinės nekrozės, plitimas į savąjį gleivinės dangalą, Ki-67 proliferacinis aktyvumas >10% naviko ląstelių populiacijos gali būti papildomi histologiniai kriterijai tikslesnei GIST prognozei nustatyti, nes yra būdingesni didelės rizikos GIST. Reikšminiai žodžiai: gastrointestininės stromos navikai, CD117, piktybinės biologinės elgsenos rizika Diagnostics of gastrointestinal stromal tumors in the National Centre of Pathology 2003–2004 Mindaugas Plečkaitis1, Ugnius Mickys2, Darius Dasevičius21 Vilnius University Emergency Hospital,Baublio str. 5, LT-08406 Vilnius, Lithuania2 National Center of Pathology,Akademijos str. 4, LT-08663 Vilnius, LithuaniaE-mails: [email protected], [email protected] Objective To review GIST cases diagnosed in 2003–2004 at the Lithuanian State Centre of Pathology, to evaluate the histological findings and to correlate histological features with the risk of malignant biological behavior. Materials and methods To evaluate the patiens’ age and sex distribution, localization, ]metastases, GIST relapses and multiple tumors. Additionally, to estimate the histological features: histological pattern (spindle, epithelioid or mixed cell type), nesting, palisading, perinuclear vacuolization of tumor cells, myxoid stroma, foci of coagulative necrosis, mucosal invasion, size, mitotic rate, immunophenotype and risk of malignant biological behavior (according to C.D.M. Fletcher) of GIST. Results Fifty GIST cases were analysed. The mean age of 36 females and 14 males was 64.38 ± 14.26 years, mediana 68 years. There were found 7 metastases (14%), 4 multiple GISTs (8%) and no one relapse case in our research. 45.7% of GISTs were localised in the stomatch, 15.2% in small intestine and 4.3% in large intestine. 46.2% of GISTs were of mixed type, 43.6% of spindle cell type and 10.3% of epithelioid type. The mean size of GISTs was 6.4 ± 5.3 cm, mediana 4.9 cm. Most of GISTs were positive to CD117 and to CD34, and one half of the tumors to a-actin. 41.7% of solitary GISTs showed a high risk of malignant biological behavior. Foci of coagulative necroses were found in 86.7% and mucosal invasion in 60% of solitary GISTs with a high risk of malignant biological behavior. Ki-67 proliferative activity >10% was characteristic of GISTs with a high risk of malignant biological behavior, whereas Ki-67 proliferative activity <5% did not correlate with the risk groups of malignant biological behavior. Conclusion The results of the research have shown that invasion of the mucosa, necrotic foci, Ki-67 proliferative activity more than 10% are characteristic of GISTs with a high risk of malignant biological behavior and should be used as additional predictors for the malignant potential of these tumors. Key words: gastrointestinal stromal tumors, CD 117, risk of malignant biological behavior


2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Anand Mohapatra ◽  
Aaron M. Potretzke ◽  
Brent A. Knight ◽  
Min Han ◽  
Robert S. Figenshau

Granulosa cell tumors (GCTs) of the testis are rare sex cord-stromal tumors that are present in both juvenile and adult subtypes. While most adult GCTs are benign, those that present with distant metastases manifest a grave prognosis. Treatments for aggressive GCTs are not well established. Options that have been employed in previous cases include retroperitoneal lymph node dissection (RPLND), radiation, chemotherapy, or a combination thereof. We describe the case of a 57-year-old man who presented with a painless left testicular mass and painful gynecomastia. Serum tumor markers (alpha fetoprotein, human chorionic gonadotropin, and lactate dehydrogenase) and computed tomography of the chest and abdomen were negative. The patient underwent left radical orchiectomy. Immunohistochemical staining was consistent with a testicular GCT. He underwent a left-template laparoscopic RPLND which revealed 2/19 positive lymph nodes. Final pathological stage was IIA. He remains free of disease 32 months after surgery.


2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e17055-e17055
Author(s):  
Syed Abdullah Javaid Bukhari ◽  
Maira Iqbal Malik ◽  
Paul Hutton ◽  
Emilio Porfiri ◽  
Mariam Jafri

e17055 Background: Testicular Sex Cord Stromal Tumors (TSCST) are rare representing approximately 5% of testicular cancers. Leydig Cell Tumours (LCT) constitute 1-3% of testicular tumours, occur commonly in third to sixth decade and are up to 50mm in diameter. Sertoli Cell Tumours(SCT) constitute less than 1%, occur commonly in the fourth decade and have an average diameter of 35mm. High-risk histological features include; tumour size, increased mitotic activity, pleomorphic nuclei, necrosis, vascular invasion and rete testis invasion. Management is surgical with no standard chemotherapy or radiotherapy in adjuvant or metastatic setting. Methods: A retrospective review of epidemiology, radiological staging, histopathological features, relapse rate and treatment in 82 patients diagnosed with TSCST between 4/12/2001 -27/12/2018 at a tertiary referral centre. Results: 2,170 testicular cancer patients were diagnosed in this period of whom 82 patients (3.8%) had TSCST. 65 patients (2.9% ) had LCT,13 patients (0.59%) had SCT, 3 patients had Unclassified SCST and 1 patient had Mixed SCST. Median patient age for LCT was 43 years, SCT was 29 years, unclassified SCST was 46 years and for mixed SCST was 49 years (age range of TSCST was 18 - 82 years). Median size of LCT was 11mm (range 4 – 28 mm) in 43 patients and of SCT was 16mm (range 7 – 60mm) in 10 patients. 79 patients (3.6% ) presented with stage 1 disease. One patient with LCT and 4 high risk histological features on initial diagnosis had local retroperitoneal recurrence 4 years later and underwent surgical resection with 1year distant disease-free survival. On distant relapse, he was treated with mitotane. One patient presented with metastatic SCT with 4 histological high risk features. He was treated with carboplatin and etoposide. Median survival in the metastatic disease was 10.5 months. Conclusions: TSCST have an excellent prognosis with over 98% of patients are free of disease post-surgery. Our relapse rate is 1.2% which is lower than previously reported and is confined to patients with high risk features. Metastatic LCT and SCT are resistant to chemotherapy with poor survival.


2021 ◽  
Vol 31 (2) ◽  
pp. 169-170
Author(s):  
R. Tyler Hillman ◽  
David Gershenson

2009 ◽  
Vol 64 (7) ◽  
pp. 455-457
Author(s):  
Gabriele Hölscher ◽  
Christoph Anthuber ◽  
Gunther Bastert ◽  
Alexander Burges ◽  
Doris Mayr ◽  
...  

2011 ◽  
Vol 122 (2) ◽  
pp. 242-245 ◽  
Author(s):  
Melissa M. Thrall ◽  
Pamela Paley ◽  
Ellen Pizer ◽  
Rochelle Garcia ◽  
Barbara A. Goff

1998 ◽  
Vol 88 (1) ◽  
pp. 116-121 ◽  
Author(s):  
David B. Clarke ◽  
Richard Leblanc ◽  
Gilles Bertrand ◽  
Gilbert R. C. Quartey ◽  
G. Jackson Snipes

✓ Meningeal melanocytomas are rare tumors of the central nervous system that are found almost exclusively in the posterior fossa and spinal cord and whose natural history is poorly defined. In this report, the authors review the clinical presentation, radiological appearance, operative findings, and histological features in two cases of meningeal melanocytoma: one cranial and one spinal. Two women, aged 21 and 30 years, were admitted to the hospital 60 years apart: the first because of progressive paraplegia and the second because of slowly progressive hearing loss. The first patient had an extradural tumor that was treated by laminectomy, subtotal resection, and postoperative radiotherapy in 1936. Her symptoms recurred 16 years later and she underwent reoperation of the residual tumor, which was found to have an intradural component. The authors' patient, who presented 60 years later, underwent plain and enhanced computerized tomography and magnetic resonance imaging that demonstrated a large posterior fossa lesion indicative of either an acoustic neuroma or a meningioma. She underwent posterior fossa decompression but only partial excision of the tumor could be accomplished because vigorous bleeding limited the extent of the resection. Surgery was followed by radiotherapy. The residual tumor enlarged despite these measures and required repeated resection 6 months later. At the second operation the tumor was much less vascular, perhaps reflecting the effects of radiotherapy, and was removed almost entirely. The patient died 6 months later from an anticoagulant-related cerebellar hemorrhage. In both cases the lesions were jet black, and histological examination revealed melanin-containing hypercellular tumors with rare mitotic figures. Meningeal melanocytomas are being diagnosed with increased frequency in parallel with improvements in neuroimaging and clarification of histological features. Clinical presentation of patients with these tumors typically occurs in their fifth decade and women are affected twice as often as men. The posterior fossa lesions can mimic acoustic neuromas and meningiomas in location and radiological appearance; however, the internal auditory canal is normal. In the spine, meningeal melanocytomas present with the clinical features of myeloradiculopathy. Diagnosis is made intraoperatively from the gross, jet-black appearance of the tumor and from histological examination. Vascularity, size, and location may render complete resection unfeasible. Because of the tumor's propensity to recur, radiotherapy has been recommended but its role remains to be elucidated.


1998 ◽  
Vol 17 (3) ◽  
pp. 266-271 ◽  
Author(s):  
Claudio Guerrieri ◽  
Birgitta Frånlund ◽  
Henric Malmström ◽  
Bernt Boeryd

1990 ◽  
Vol 9 (4) ◽  
pp. 352-362 ◽  
Author(s):  
Hironobu Sasano ◽  
J Ian Mason ◽  
Etsuko Sasaki ◽  
Akira Yajima ◽  
Noriko Kimura ◽  
...  

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