Endometrial stromal sarcoma in a 20-year-old woman

Endometrial stromal sarcoma (ESS) is an uncommon and challenging condition comprising 10% of all uterine sarcomas and found in women 42–58 years of age. ESS is difficult to diagnose in young women as it masquerades as a leiomyoma. We report this tumour in a 20-year-old woman presenting with heavy and prolonged menses and urinary retention. She was not sexually active and did not give consent for pelvic examination. A preoperative diagnosis of a submucous leiomyoma with an adnexal mass was made. At laparotomy, the leiomyoma was found to be wedged between the cervix and the vagina, and was removed vaginally. A 5–6 cm retroperitoneal mass was adherent to the right pelvic wall, which was also removed. Histopathology of both specimens revealed ESS. The final diagnosis according to the International Federation of Gynaecology and Obstetrics classification was stage IV ESS. After oncology consult, she was referred for chemotherapy. She is now on follow-up.

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Endometrial Stromal Sarcoma Recurrence in the Caecum

Case Reports in Surgery ◽

10.1155/2018/9139281 ◽

2018 ◽

Vol 2018 ◽

pp. 1-4

Author(s):

Nisar A. Chowdri ◽

Asif Mehraj ◽

Fazl Q. Parray ◽

Mudassir A. Khan ◽

Masood A. Laharwal ◽

...

Keyword(s):

Rare Case ◽

Endometrial Stromal Sarcoma ◽

Total Abdominal Hysterectomy ◽

Abdominal Hysterectomy ◽

Low Grade ◽

Lower Abdomen ◽

Polypoidal Lesion ◽

Stromal Sarcoma ◽

The Right

Endometrial stromal sarcomas of uterus are quite rare. Most of the recurrences in these tumors are seen in the pelvis. However, extrapelvic recurrences and metastases to other parts are quite unusual. Here, we are reporting a rare case of caecal recurrence of endometrial stromal sarcoma. Case Report. A 52-year-old female presented to us with pain and lump in the right lower abdomen. The patient was earlier subjected to total abdominal hysterectomy with bilateral salpingo-oophorectomy (TAHBSO) for low-grade endometrial stromal sarcoma. Postoperatively patient received radiotherapy but no hormone therapy. After 10 years of follow up patient presented with a polypoidal lesion in the caecum. Patient was evaluated fully and subjected to resection of this polypoidal lesion, which proved out to be high-grade endometrial stromal sarcoma. Conclusion. Recurrence of endometrial stromal sarcoma in the caecum is very rare. However, this entity needs to be kept in mind for differential diagnosis of a caecal mass. Recurrence in such cases may present quite late.

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Clinical outcomes of uterine sarcomas: results from 14 years worth of experience in the Kinki district in Japan (1990–2003)

International Journal of Gynecological Cancer ◽

10.1136/ijgc-00009577-200605000-00062 ◽

2006 ◽

Vol 16 (3) ◽

pp. 1358-1363

Author(s):

K. Kokawa ◽

K. Nishiyama ◽

M. Ikeuchi ◽

Y. Ihara ◽

N. Akamatsu ◽

...

Keyword(s):

Clinical Outcomes ◽

Early Stage ◽

Disease Free Survival ◽

Stage Iv ◽

Endometrial Stromal Sarcoma ◽

Uterine Sarcomas ◽

Stromal Sarcoma ◽

Younger Age ◽

Histologic Types ◽

Disease Free

To review clinical outcomes and therapeutic varieties, we were invited to submit data from the patients who were treated for uterine sarcomas in Japan from 1990 to 2003. Uterine sarcomas were defined as leiomyosarcoma (LMS), endometrial stromal sarcoma (ESS), and carcinosarcoma (CS). Of a total of 97 patients, 36 (37.1%) were diagnosed with LMS of the uterine corpus, 15 (15.5%) with ESS, 46 (47.4%) with CS. Median age at diagnosis was 59 (21–85) years. Clinical stages based on FIGO were 41 (42.3%) with stage I disease, 6 (6.2%) with staged II, 34 (35.1%) with stage III, and 16 (16.5%) with stage IV. The median follow-up period for all patients was 13 (1–108) months and median disease-free period was 9 (0–96) months. The 1-year survival rate and disease-free survival (DFS) rate were calculated in patients with all sarcomas (overall survival [OAS], 61.3%; DFS, 46.6%). Statistical analysis showed that younger age (less than 50 years), early stage (stages I and II), and surgical procedure (extended hysterectomy [EH] and radical hysterectomy [RH]) were associated with significantly better OAS. Histologic types did not affect the survival period. In conclusion, aggressive surgery including EH or RH at the time of initial operation offers the possibility of prolonged survival.

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Oral Abstract

10.1055/s-0039-1685335 ◽

2016 ◽

Author(s):

Dharma Ram

Keyword(s):

Survival Data ◽

Eligible Patient ◽

Stage Iv ◽

Endometrial Stromal Sarcoma ◽

Uterine Sarcoma ◽

Low Grade ◽

Uterine Leiomyosarcoma ◽

High Grade ◽

Lost To Follow Up

Introduction: Uterine sarcoma accounts for nearly 3% of all uterine malignancies. They have 4 major pathology includes endometrial stromal sarcoma high grade, ESS low grade, uterine leiomyosarcoma (uLMS) and undifferentiated uterine sarcoma (UUS). Recent WHO classification 2014, recognizes low grade ESS and high grade ESS as distinct entity. They differ from endometrial carcinoma in their aggressive nature and poor prognosis. We review our database and found total 44 eligible patient treated at our institute. Materials and Methods: Its retrospective analysis of computer based database of our institute from January 2009 to December 2015. We analyzed demographic, pathological, treatment and survival data. Results: Total 44 patient treated for uterine sarcoma at our institute. Among these 16 were operated at our institute during study period. Here we reporting results of operated patients at our institute. The histological diagnosis LMS (5/16), ESS-L (4/16), MMMT (3/16), UUS (3/16) and ESS-H (1/16). Stage distribution was stage I, (6/16) stage II, (5/16) stage III, (3/16) stage IV, (0/16) and unknown stage (2/16). Two patients underwent completion surgery for outside myomectomy. The adjuvant treatment was CT in 3/16, CT with RT in 7/16, HT in 4/16 and one lost to follow up with one was put on observation. Median follow up is 30 month with 14 patients alive and one lost to follow up. At last follow up 4 patients alive with metastatic disease and 10 patients alive with no evidence of disease. Conclusion: Uterine sarcoma are uncommon disease with

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JAZF1–SUZ12 endometrial stromal sarcoma forming subserosal masses with extraordinary uptake of fluorodeoxyglucose on positron emission tomography: a case report

Diagnostic Pathology ◽

10.1186/s13000-019-0897-y ◽

2019 ◽

Vol 14 (1) ◽

Author(s):

Koto Fujiishi ◽

Shigenori Nagata ◽

Rieko Kano ◽

Chiaki Kubo ◽

Maasa Shirayanagi ◽

...

Keyword(s):

Positron Emission Tomography ◽

Endometrial Stromal Sarcoma ◽

Emission Tomography ◽

Low Grade ◽

Neoplastic Cells ◽

Pelvic Magnetic Resonance Imaging ◽

Stromal Sarcoma ◽

Positron Emission ◽

Mesenchymal Neoplasm ◽

The Right

Abstract Background Low-grade endometrial stromal sarcoma (ESS) is rare mesenchymal neoplasm, recently specified as harboring JAZF1–SUZ12 rearrangement. Typical JAZF1–SUZ12 ESS is slow growing, in which high uptake of fluorodeoxyglucose (FDG) on positron emission tomography (PET) and subserosal masses are quite unusual. Case presentation A 69-year-old Japanese woman complained of urinary incontinence. Pelvic magnetic resonance imaging showed uterine lesions composed of (1) a 9 × 8 × 7-cm mass protruding from the right-anterior wall, (2) a 4.5-cm mass attached to the right-posterior wall, and (3) a 6.5-cm intramural mass in the fundus. FDG-PET demonstrated maximum standardized uptake value of 13.28 confined to the two subserosal masses (1 & 2) in contrast to no uptake of the intramural mass (3). She was diagnosed with a high-grade uterine sarcoma concomitant with leiomyomas and underwent total hysterectomy with bilateral salpingo-oophorectomy and pelvic lymphadenectomy. The removed uterus had three tumors—two in the right-anterior and right-posterior subserosa, respectively, and the remaining in the fundal myometrium. Microscopically, the three tumors shared morphologic features characterized by neoplastic cells similar to proliferative-phase endometrial stromal cells, in which neither round-cell component, pleomorphism, nor high mitotic activity was recognized. Nuclear cyclin D1 immunostaining was identified 50% of neoplastic cells in the two subserosal tumors (1 &2) whereas < 1% positive cells in the intramural component (3). Reverse transcriptase-polymerase chain reaction showed the same-sized electrophoretic bands indicating JAZF1–SUZ12 gene fusion shared by the three uterine tumors and a focal tumor extension into the extrauterine vein. The patient is alive without evidence of recurrence at 14 months after surgery. Conclusions Pathologists and clinicians should not exclude the possibility of JAZF1–SUZ12 ESS even when uterine subserosal masses demonstrate extraordinary FDG uptake on PET. Molecular analysis is helpful for diagnostic confirmation of JAZF1–SUZ12 ESS with a complex growth pattern.

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A Clinical and Pathologic Study of 34 Sarcomas of the Uterus

Tumori Journal ◽

10.1177/030089168106700411 ◽

1981 ◽

Vol 67 (4) ◽

pp. 341-348 ◽

Cited By ~ 3

Author(s):

Rado Kenda ◽

Giuseppe De Palo ◽

Salvatore Andreola ◽

Gaetano Bandieramonte ◽

Giovanni Lupi ◽

...

Keyword(s):

Clinical Trials ◽

Endometrial Stromal Sarcoma ◽

Controlled Clinical Trials ◽

Relapse Free Survival ◽

Histologic Subtype ◽

Uterine Sarcomas ◽

Free Survival ◽

Stromal Sarcoma ◽

Pathologic Features ◽

History Of

The clinical and pathologic features of 34 uterine sarcomas were studied to determine the natural history of the disease. Sixteen patients had leiomyosarcoma, five mixed mesodermal sarcoma, ten endometrial stromal sarcoma, two carcinosarcoma and one endolymphatic stromal myosis. The patients were treated without an unique protocol. At 3 years the actuarial relapse-free survival was 53.6 %: 68.4 % in stage I-II patients and 22.2 % in stage III-IV patients. As regards the histologic subtype mixed mesodermal sarcomas had the best prognosis; endometrial stromal sarcomas the worst. The necessity of a uniform clinical and histologic classification as well as the importance of controlled clinical trials are pointed out.

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Isobolographic Analysis Demonstrates the Additive and Synergistic Effects of Gemcitabine Combined with Fucoidan in Uterine Sarcomas and Carcinosarcoma Cells

Cancers ◽

10.3390/cancers12010107 ◽

2019 ◽

Vol 12 (1) ◽

pp. 107 ◽

Cited By ~ 3

Author(s):

Marcin Bobiński ◽

Karolina Okła ◽

Jarogniew Łuszczki ◽

Wiesława Bednarek ◽

Anna Wawruszak ◽

...

Keyword(s):

Cell Cycle ◽

Synergistic Effect ◽

Cell Line ◽

Cell Lines ◽

Combined Treatment ◽

Endometrial Stromal Sarcoma ◽

Uterine Leiomyosarcoma ◽

Uterine Sarcomas ◽

Isobolographic Analysis ◽

Stromal Sarcoma

Background: Uterine sarcomas and carcinosarcoma are associated with unfavorable prognosis. The regimens that are used in chemotherapy are associated with high incidence of side effects and usually do not significantly increase patients’ survival rates. In this study we investigated the activity and interactions between gemcitabine and fucoidan, the natural compound known for its anti-tumor properties, in human sarcomas and carcinosarcoma cell models. Methods: SK-UT-1, SK-UT1-B (carcinosarcoma), MES-SA (leiomyosarcoma), and ESS-1 (endometrial stromal sarcoma) cell lines were used for the experiments. Cells were incubated in the presence of gemcitabine, fucoidan, and mixtures, after the incubation the MTT tests were performed. In order to assess the interactions between tested compounds isobolographic analysis was performed. Additional assessments of apoptosis and cell cycle were done. Results: Additive effect of combined treatment with gemcitabine and fucoidan was observed in ESS-1 and SK-UT-1 cell line. Although the supra-additive (synergistic) effect noticed in SK-UT-1B cell line. It was not possible to determine the interactions of fucoidan and gemcitabine in MES-SA cell line due to insufficient response to treatment. Addition of fucoidan to gemcitabine enhances its proapoptotic activity, what was observed especially in ESS-1 and SK-UT-1B cell lines. The arrest of cell cycle induced by mixture of gemcitabine and fucoidan, superior comparing gemcitabine alone was observed in SK-UT-1B. Conclusions: Obtained data showed that a combination of fucoidan and gemcitabine in uterine endometrial stromal sarcoma and carcinosarcoma cell lines has additive or even synergistic effect in decreasing cell viability. Furthermore, this drug combination induces apoptosis and arrest of cell cycle. The resistance of uterine leiomyosarcoma cell line, justifies searching for other drugs combinations to improve therapy efficacy.

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Treatment of Stage IV “High-Grade” Endometrial Stromal Sarcoma with Ifosfamide, Adriamycin, and Cisplatin

Gynecologic Oncology ◽

10.1006/gyno.1996.4537 ◽

1997 ◽

Vol 64 (2) ◽

pp. 265-269 ◽

Cited By ~ 22

Author(s):

Takaharu Yamawaki ◽

Yoshio Shimizu ◽

Katsuhiko Hasumi

Keyword(s):

Stage Iv ◽

Endometrial Stromal Sarcoma ◽

High Grade ◽

Stromal Sarcoma

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Uterine Sarcoma and Aromatase Inhibitors: Tom Baker Cancer Centre Experience and Review of the Literature

International Journal of Gynecological Cancer ◽

10.1097/igc.0b013e31825b7de8 ◽

2012 ◽

Vol 22 (6) ◽

pp. 1006-1012 ◽

Cited By ~ 23

Author(s):

Alon D. Altman ◽

Gregg S. Nelson ◽

Pamela Chu ◽

Jill Nation ◽

Prafull Ghatage

Keyword(s):

Partial Response ◽

Aromatase Inhibitors ◽

Retrospective Studies ◽

Response Rate ◽

Case Reports ◽

Endometrial Stromal Sarcoma ◽

Uterine Sarcoma ◽

Uterine Sarcomas ◽

Cancer Centre ◽

Stromal Sarcoma

ObjectivesUterine sarcomas are a rare group of mesenchymal tumors with a poor prognosis and aggressive biology. Standard treatment involves surgical staging. The role of further adjuvant treatment is unclear. The goals of this study were to determine the response rates to treatment of patients with uterine sarcomas and to review the currently available literature on the use of aromatase inhibitors (AIs).Materials and MethodsWe performed a retrospective analysis on all patients with uterine sarcoma treated with an AI between 2000 and 2010 at the Tom Baker Cancer Centre in Calgary, Alberta.ResultsFour patients with endometrial stromal sarcoma and 3 patients with leiomyosarcoma received treatment with an AI. A literature search resulted in 10 case reports and 4 retrospective studies of patients with endometrial stromal sarcoma and 1 case report and 2 retrospective studies of patients with leiomyosarcoma. On the basis of the available literature, combined with the current findings, the overall response rate of endometrial stromal sarcoma to AIs is 67% (complete response of 7% and partial response of 60%), and the partial response rate of leiomyosarcoma to AIs is 11%, with no reported complete responses.ConclusionsAromatase inhibitors are a well-tolerated class of medications that are effective in the treatment of endometrial stromal sarcomas. These medications may also have a role to help stabilize disease progression in the treatment of leiomyosarcoma. More large, prospective, multicentered trials will be needed to clarify this issue.

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Clinical Characteristics of Nodular Fasciitis of Ear in Children

10.21203/rs.3.rs-484827/v1 ◽

2021 ◽

Author(s):

Xiaoxu Wang ◽

Wei Liu ◽

Lejian He ◽

Min Chen ◽

Jianbo Shao ◽

...

Keyword(s):

Surgical Resection ◽

Clinical Characteristics ◽

Clinical Manifestations ◽

Neck Surgery ◽

Final Diagnosis ◽

Diagnosis And Treatment ◽

Nodular Fasciitis ◽

Gene Test ◽

The Right

Abstract Purpose Summarized the clinical characteristics and diagnosis and treatment process of three cases of nodular fasciitis of ear, to provide a basis for clinical diagnosis and treatment. Methods Reviewed the clinical manifestations, images, pathology, treatment and postoperative follow up results of three cases of pediatric nodular fasciitis in the Department of Otorhinolaryngology, Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University from 2018 to 2020. Results The average age at diagnosis were 24 months, with two girls and a boy. Two lesions were found in the left ear and one in the right ear. All cases had a history of biopsy before surgery. Two of three cases showed a sign of rapid growth after biopsy and three of which were ineffective in anti-inflammatory treatment. FISH test for USP6 were performed in two of the three cases with positive results. Three lesions show a hypointensity or isointensity on T1-weighted MRI and a heterogeneous hyperintensity on T2-weighted MRI. ‘‘Fascial tail’’ sign was found on image of all three cases. All lesions underwent surgical resection. Follow-up showed no recurrence and had an intact ear appearance. Conclusion The early misdiagnosis rate of nodular fasciitis of the ear is high. Combine clinical features with imaging findings may improve the accuracy of preoperative diagnosis. Besides the appearance of pathology, USP6 gene test is also an important tool in the diagnosis. The final diagnosis should be based on comprehensive assessment. Complete surgical resection can prevent recurrence.

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Colorectal cancers in United Arab Emirates nationals: The Sheik Khalifa Medical Center's experience.

Journal of Clinical Oncology ◽

10.1200/jco.2012.30.4_suppl.442 ◽

2012 ◽

Vol 30 (4_suppl) ◽

pp. 442-442 ◽

Cited By ~ 1

Author(s):

Nidal Dehni ◽

Ali Al Hassani ◽

Abdulrahman Nimeri

Keyword(s):

United Arab Emirates ◽

Screening Program ◽

Stage Iv ◽

Emergency Operation ◽

Final Diagnosis ◽

Tumor Location ◽

Colorectal Cancers ◽

Number Of Patients ◽

Co Morbidity

442 Background: Previous reports suggested that colorectal cancers (CRC) appear at younger age in the United Arabs Emirates (UAE). However, these reports included nationals and expatriates in their analysis with heterogeneous population leading to biased analysis. our objective was to determine age and stage of disease in newly diagnosed UEA nationals with CRC treated at one major referral hospital in Abu Dhabi (SKMC) Methods: Charts of all patients diagnosed and/ or treated for CRC at SKMC between January 2000 and May 2011 were reviewed. Ultimately, only UEA nationals with diagnosis of adenocarcinoma of the colon and rectum were retained for further analysis. Results: Two hundreds six patients were diagnosed at SKMC as having colon or rectal carcinomas. Ninety two were expatriates and in 10 out of 113 nationals, the final diagnosis was squamous or adenosquamous carcinoma leaving 103 patients forming the population of this study. Median age was 57(10-100 years), with 59 men. The patient’s condition necessitated emergency operation in 38 and 15 either refused or were unfit for treatment mainly because of very advanced disease or severe co morbidity. The tumor location was: sigmoid: 46%, rectum: 33:, right colon :17% and 4% for descending and transverse colon. The stage of the disease was I; 4 pts, II: 16 pts, III 25 pts, IV in 49 pts and undetermined in 7. Resection was curative for 46 pts, palliative in 45, unknown in 10 and 2 refused surgery. Fifty one patients had their treatment essentially at SKMC and the others were treated both at SKMC and abroad. Average follow-up was 2 years and at last FU 39 were confirmed deceased, 29 were alive and the outcome in the remaining was unknown. Conclusions: UAE nationals with CRC presenting to our facility have relatively young age but presented with stage IV disease in half of the cases. Screening program for this population is warranted. Because a substantial number of patients had had their treatment abroad, rigorous follow-up and cancer outcome assessment was unreliable.

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