Improvement of Survival in Sex Cord Stromal Tumors: An Observational Study With More Than 25 Years Follow-Up

Abstract Objective Limited data are available concerning long-term results of imatinib therapy in patients with advanced gastrointestinal stromal tumors. We aimed to clarify the long-term outcomes of imatinib therapy in Japanese patients with advanced gastrointestinal stromal tumors. Methods A prospective, observational study of imatinib therapy for unresectable and metastatic gastrointestinal stromal tumors was conducted in our institution. Imatinib was initiated at a dose of 400 mg daily and continued until disease progression. Safety, efficacy and long-term tolerability and survival were evaluated in an intent-to-treat population. The median follow-up period in this study was 68 months. Results Seventy patients were enrolled between December 2001 and December 2009. Treatment-related Grade 3/4 adverse events occurred in 49 patients (70.0%). Although 14 patients required adverse effect management with hospitalization, only 5 patients (7.1%) withdrew from the treatment owing to imatinib intolerance. The tumor response and clinical benefit rates were 61.4 and 85.7%, respectively. Thirty-seven patients (52.9%) maintained the treatment at 400 mg daily imatinib, whereas 33 patients (47.1%) had their dose reduced to 300 mg daily or less. The overall survival rate at 5 years was 60.9% and the median survival time was 70 months. The median progression-free survival time of all the 70 enrolled patients was 30 months. Seven patients (10.0%) suffered from second malignancies, including three patients with genitourinary carcinomas. Conclusions Despite the need for dose reduction, the long-term results of imatinib therapy for advanced gastrointestinal stromal tumors were good in Japanese patients. Physicians should pay attention to the occurrence of second malignancies during imatinib therapy for gastrointestinal stromal tumor patients.

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Hepatoid Carcinoma of the Ovary: Clinical, Histopathologic, and Immunophenotypic Features

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2017-0485-rs ◽

2019 ◽

Vol 143 (7) ◽

pp. 883-889 ◽

Cited By ~ 3

Author(s):

Andres M. Acosta ◽

Michael R. Pins

Keyword(s):

Gonadal Dysgenesis ◽

Residual Disease ◽

Giant Cells ◽

Nuclear Pleomorphism ◽

Stromal Tumors ◽

Hepatoid Carcinoma ◽

Metastatic Hepatocellular Carcinoma ◽

Carcinoma Of The Ovary ◽

Sex Cord Stromal Tumors

Hepatoid carcinoma of the ovary (HCO) is a rare malignant tumor of uncertain histogenesis that was first described by Ishikura and Scully in the late 1980s. Unlike hepatoid yolk sac tumor (HYST), one of its main differential diagnoses, HCO usually presents in perimenopausal and postmenopausal women without gonadal dysgenesis. Most cases show advanced local disease at initial presentation, with diffuse intraperitoneal dissemination. Despite aggressive treatment, including surgery and adjuvant chemotherapy, 61.5% of patients either die of the disease (11 of 26; 42.3%) or are alive with recurrent or residual disease (5 of 26; 19.2%) after a median follow-up of 11.5 months (range, 1–60 months). Most HCOs are solid, with high-grade histology, significant nuclear pleomorphism, scattered giant cells, and a high mitotic index. Their immunophenotype is defined by the expression of broad-spectrum cytokeratins, α-fetoprotein, and hepatocellular antigens with absence of sex cord and germ cell markers. Although immunohistochemistry can be very helpful to distinguish between sex cord–stromal tumors and HCO, differentiation of the latter from HYST, metastatic hepatocellular carcinoma, and metastatic gastrointestinal tumors with hepatoid phenotype requires integration of clinical, radiologic, and pathologic information.

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Ovarian Sex Cord–Stromal Tumors in Children and Adolescents

Journal of Clinical Oncology ◽

10.1200/jco.2003.05.038 ◽

2003 ◽

Vol 21 (12) ◽

pp. 2357-2363 ◽

Cited By ~ 67

Author(s):

D.T. Schneider ◽

G. Calaminus ◽

R. Wessalowksi ◽

R. Pathmanathan ◽

B. Selle ◽

...

Keyword(s):

Malignant Ascites ◽

Stage Ii ◽

Event Free Survival ◽

Free Survival ◽

Stromal Tumors ◽

Gynecology And Obstetrics ◽

Diagnostic Standards ◽

Stage Ia ◽

Sex Cord Stromal Tumors

Purpose: To develop diagnostic standards and a risk-adapted therapeutic strategy for ovarian sex cord–stromal tumors (OSCST). Patients and Methods: Fifty-four patients were prospectively enrolled as follow-up patients onto the German Maligne Keimzelltumoren protocols. Surgical protocols and histopathology were reviewed centrally (53 patients with complete data). Surgery included ovariectomy in 18 patients, salpingo-ovariectomy in 34 patients, and hysterectomy in one patient. Patients with stage IA tumors were followed-up at regular intervals, whereas nine patients with stage IC and six patients with stage II to III tumors were treated with cisplatin-based chemotherapy. Results: International Federation of Gynecology and Obstetrics stage was IA in 27 patients, IC in 21 patients, II in three patients, and III in three patients. After a median follow-up of 59 months (range, 6 to 193 months), event-free survival ± SD was 0.86 ± 0.05 (47 of 54 patients) and overall survival was 0.89 ± 0.05 (49 of 54 patients). Prognosis correlated with stage (event-free survival ± SD: IA, 1.0 [27 of 27 patients]; IC, 0.76 ± 0.09 [16 of 21 patients]; and II/III, 0.67 ± 0.19 [four of six patients]; P = .02). Ten of 15 patients treated with chemotherapy, including four of six stage II to III patients, are alive after a median follow-up of 33 months. Conclusion: On the basis of a standardized clinical and histopathologic assessment, risk-adapted therapeutic strategies for OSCST can be evaluated. Considering our experience, we would recommend that stage IA tumors be followed up at regular intervals, whereas we would recommend cisplatin-based chemotherapy in stage IC tumors with preoperative rupture or malignant ascites, especially those with high mitotic activity. Finally, cisplatin-based chemotherapy also seems to be effective in advanced-stage tumors.

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The Activity of Taxanes in the Treatment of Sex Cord-Stromal Ovarian Tumors

Journal of Clinical Oncology ◽

10.1200/jco.2004.12.074 ◽

2004 ◽

Vol 22 (17) ◽

pp. 3517-3523 ◽

Cited By ~ 60

Author(s):

Jubilee Brown ◽

Hyun S. Shvartsman ◽

Michael T. Deavers ◽

Thomas W. Burke ◽

Mark F. Munsell ◽

...

Keyword(s):

Progression Free Survival ◽

Complete Response ◽

Cancer Center ◽

First Line ◽

Stromal Tumors ◽

Number Of Patients ◽

Measurable Disease ◽

Line Setting ◽

Sex Cord Stromal Tumors

Purpose To determine the efficacy and side effects of taxanes, with or without platinum, for the treatment of sex cord-stromal tumors of the ovary. Patients and Methods We conducted a retrospective review of all patients seen from 1985 to 2002 at The University of Texas M.D. Anderson Cancer Center with ovarian sex cord-stromal tumors. Eligible patients underwent pathology confirmation and clinical evaluation at M.D. Anderson and received a taxane for initial or recurrent disease. Results Of 222 patients identified, 44 were eligible for analysis. For nine patients treated in the first-line adjuvant setting, median progression-free survival (PFS) was not reached at 51 months. Of two patients treated for measurable disease in the first-line setting, one had a complete response. Median PFS was 34.3 months; median overall survival (OS) was not reached. Median follow-up was 90.3 months (range, 39.4 to 140.5 months). Response rate for 30 patients treated with a taxane ± platinum for recurrent, measurable disease was 42%. Median PFS was 19.6 months; median OS was not reached. Median follow-up was 100.7 months (range, 8.1 to 361.3 months). The presence of platinum correlated with response in the recurrent, measurable disease setting. The number of patients was insufficient to detect relative efficacy of paclitaxel and docetaxel. Adverse effects of paclitaxel included neutropenia (n = 6), anemia (n = 1), thrombocytopenia (n = 1), myelodysplasia (n = 1), and hypersensitivity (n = 1). Conclusion Taxanes seem to be active agents in the treatment of patients with sex cord-stromal tumors of the ovary. The combination of taxanes with platinum in the treatment of this disease deserves additional investigation.

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A rare case of virilizing adult granulosa cell tumor

Journal of Nepal Medical Association ◽

10.31729/jnma.806 ◽

1970 ◽

Vol 39 (136) ◽

pp. 351-353

Author(s):

Kaveeta Dawaka ◽

A Gupta ◽

S Joshi ◽

S Bajpai ◽

H S Basi

Keyword(s):

Granulosa Cell ◽

Ovarian Tumor ◽

Granulosa Cell Tumor ◽

Cell Tumor ◽

Stromal Tumors ◽

Cystic Tumors ◽

History Of ◽

Disproportionate Number ◽

Sex Cord Stromal Tumors

A twenty eight year old lady, married for two years with no issue, presented at WesternRegional Hospital Pokhara on 8th July, 1999 with a history of amenorrhea for 1½years, lump abdomen 1 year, hoarseness of voice for 1 year and signs of hirsutism forthe same duration. She was taken up for laparotomy wherein a left ovariectomy withsalpingectomy was done and a large cystic ovarian tumor was removed. Post operativeperiod was uneventful and she was advised follow up. The histopathological diagnosiswas Adult Granulosa Cell Tumor. What made this case interesting is that mostGranulosa Cell Tumors (about 3/4th) are estrogenic or feminizing, but rare examples,including a disproportionate number of large thin walled cystic tumors are androgenic.This was one of those rare virilizing tumors with classical features of infertility,amenorrhea, hoarseness of voice, clitiromegaly, and breast atrophy, but we could notestimate testosterone levels due to lack of facilities available.Key Words: Granulosa Cell Tumor, Virilizing Ovarian Tumors, Sex Cord stromal tumors.

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Initial experiences utilizing perfusion CT in the follow-up of patients with gastrointestinal stromal tumors under imatinib mesylate treatment

RöFo - Fortschritte auf dem Gebiet der Röntgenstrahlen und der bildgebenden Verfahren ◽

10.1055/s-0028-1085909 ◽

2008 ◽

Vol 180 (09) ◽

Author(s):

F Berger ◽

M Hittinger ◽

M Schlemmer ◽

T Saam ◽

K Nikolaou ◽

...

Keyword(s):

Imatinib Mesylate ◽

Gastrointestinal Stromal Tumors ◽

Perfusion Ct ◽

Stromal Tumors

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Multislice imaging of gastrointestinal stromal tumors

10.32512/jmr.1.1.2018/3.14 ◽

2018 ◽

pp. 3-14

Keyword(s):

Computed Tomography ◽

Key Words ◽

Gold Standard ◽

Gastrointestinal Stromal Tumors ◽

Mesenchymal Tumors ◽

Stromal Tumors ◽

Preferential Localization ◽

Multislice Imaging ◽

Computed Tomography Diagnosis

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the digestive tract (1%). These tumors express the CD 117 in 95% of cases. The stomach is the preferential localization (70%). Diagnosis is difficult and sometimes late. Progress of imaging has greatly improved the management and the prognosis. Computed tomography (CT) is the gold standard for diagnosis, staging, and treatment follow-up. The increasing recognition of GIST’s histopathology and the prolonged survival revealed some suggestive imaging aspects. Key words: gastro-intestinal stromal tumors; computed tomography; diagnosis

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