Uterine sarcomas

Uterine sarcomas: Magnetic resonance presentation and clinical context - a retrospective study in an oncological medical center. Poster No.:

C-0766

Congress:

ECR 2015

Type:

Educational Exhibit

Authors:

A. Batista, T. M. Cunha; Lisbon/PT

Keywords:

Pathology, Education and training, Cancer, Diagnostic procedure, Contrast agent-intravenous, Comparative studies, RIS, PACS, MR, Oncology, Management, Genital / Reproductive system female

DOI:

10.1594/ecr2015/C-0766

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Learning objectives •

To discuss the most common symptoms and signs in a uterine sarcoma presentation as well as its different histological subtypes.

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To evidence the frequently unspecific clinical presentation of uterine sarcomas and the consequent evident relevance of magnetic resonance imaging (MRI) in its assessment, namely in pre-operative detection and staging, as well in directing appropriate management.

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To retrospectively evaluate 21 uterine sarcoma cases admitted to our institution, primarily correlating clinical presentation, histological subtype and magnetic resonance imaging distinctive features.

Background Uterine sarcomas are a rare group of tumours, with frequent aggressive behaviour, responsible for approximately 1% of female genital tract malignancies and 3% to 7% of all uterine cancers [1]. Since the new (2009) International Federation of Gynecology and Obstetrics (FIGO) criteria (Table 1) [2], they have been divided in leiomyosarcomas (originating in myometrial smooth muscle), endometrial stromal sarcomas and undifferentiated endometrial sarcomas (from endometrial stroma) and adenosarcoma, with endometrial or myometrial origin, formed from both benign epithelial cells and a sarcomatous component (carcinosarcoma has been re-classified as a dedifferentiated/metaplastic endometrial carcinoma) [3].

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Table 1: FIGO staging for uterine sarcomas.

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References: FIGO Committee on Gynecologic Oncology. FIGO staging for uterine sarcomas. Int J Gynaecol Obstet 2009; 106:277 The clinical presentation of uterine sarcomas is non-specific, classically presenting as a rapid growth uterine tumour, which may be accompanied with vaginal bleeding and abdominal or pelvic pain. Its peak incidence is between 50 and 65 years of age [4]. Clinical distinction between different types of uterine sarcoma and other uterine neoplasms, namely endometrial carcinoma and leiomyomas, is impracticable, thus being imaging evaluation, primarily by MRI, of particular relevance [5]. •

Leiomyosarcomas are the most common histological variant, accountable for one third of uterine sarcomas, most arising de novo and rarely (0.2%) developing from sarcomatous transformation in a benign leiomyoma [6]. They have poor prognosis, on MRI evaluation frequently presenting with heterogeneous hypointensity on T1-weighted images, irregular and ill-defined margins, high T2-weighted signal and early post gadolinium heterogeneous enhancement. Early hematogenous spread to liver and lungs is common [5].

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Fig. 1: MRI at 1,5T. Leiomyosarcoma in a 51-year-old woman. Large uterine mass with ill-defined margins and heterogeneous T1-weighting, presenting with high signal areas translating haemorrhagic transformation (white arrow).

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References: Department of Radiology, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisbon, Portugal

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Fig. 2: MRI at 1,5T. Leiomyosarcoma in a 51-year-old woman. Heterogeneous uterine mass with central area of high signal representing necrosis, in T2-weighted MR image. The endometrial line is compressed anteriorly by the tumour (white arrow).

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Fig. 3: MRI at 1,5T. Leiomyosarcoma in a 51-year-old woman. T1-weighted fat saturated image after gadolinium administration, in arterial phase. Tumour areas of high signal corresponding to hypervascular neoplastic tissue (white arrow), low signal

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in necrotic regions (star) and intermediate signal in mostly haemorrhagic areas (as evident in previous T1-weighted same section image). References: Department of Radiology, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisbon, Portugal

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Fig. 4: MRI at 1,5T. Leiomyosarcoma in a 53-year-old woman. DWI at b-1000 revealing evident mass hypercellularity with prominent high signal.

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Fig. 5: MRI at 1,5T. Leiomyosarcoma in a 53-year-old woman. True protonic restriction due to highly cellular tumour can be confirmed referring to low diffusion as stated by the ADC map, in DWI.

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References: Department of Radiology, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisbon, Portugal •

Endometrial stromal sarcomas are relatively indolent, associated with long term survival, frequently appearing as polypoid endometrial mass, with low signal on T1-weighted images and heterogeneously increased high T2 signal. These tumours have a tendency for lymphatic and vascular invasion, with visible nodular extension into the myometrium ("bag of worms" appearance). They have moderate and commonly heterogeneous enhancement (in this aspect clearly differentiating themselves of endometrial carcinoma, frequently hypovascular) [7].

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Fig. 6: MRI at 1,5T. Endometrial stromal sarcoma in an 83-year-old woman. Large infiltrating tumour that extends from the cervix to the superior third of the vagina, with

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some peripheral nodularity and demonstrating high T2-weighted signal due to tumour necrosis. References: Department of Radiology, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisbon, Portugal

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Fig. 7: MRI at 1,5T. Endometrial stromal sarcoma in an 83-year-old woman. Evident areas of tumour hypervascular tissue (in stark contrast to commonly hypovascular endometrial carcinoma).

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Fig. 8: MRI at 1,5T. Endometrial stromal sarcoma in an 83-year-old female patient. Protonic mobility restriction due to hypercellular tissue with high b-600 signal.

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Fig. 9: MRI at 1,5T. Endometrial stromal sarcoma in an 83-year-old woman. Hypercellular tumoral tissue with low ADC. References: Department of Radiology, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisbon, Portugal

Fig. 10: MRI at 1,5T. Sagittal T2-weighted image shows an endometrial stromal sarcoma in a 64-year-old woman, with typical nodular "bag of worms" invasion of adjacent myometrium (due to vascular and lymphatic spread, with normal myometrium represented by linear strands in hyposignal) and bladder.

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References: Department of Radiology, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisbon, Portugal •

In contrast, undifferentiated endometrial sarcomas are very aggressive, with a 5-year survival rate of 25-55%, marked T1 and T2-weighted heterogeneity, hyperenhancement and more aggressive and destructive expansion into neighbouring myometrium [8].

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Fig. 11: MRI at 1,5T. Undifferentiated endometrial sarcoma in a 36-year-old woman. On sagittal T1-weighted image after gadolinium administration, the lesion shows intense and heterogeneous contrast uptake, interspersed areas of low signal necrosis and irregular nodular margins. References: Department of Radiology, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisbon, Portugal •

Adenosarcomas have typical slow-growth, with a 5-year survival rate above 80%, frequently presenting as a well-demarcated polypoid multiseptated cystic mass protruding through the cervical os [9].

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Fig. 12: MRI at 1,5T. Adenosarcoma in a 76-year-old woman. Sagittal T2-weghted image showing a very large polypoid uterine multicystic mass protruding into the cervical os. References: Department of Radiology, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisbon, Portugal Diffusion-weighted MRI imaging plays a well-known role in affirming hypercellularity, particularly in high grade histological types, demonstrating high signal intensity on b-1000 images with low apparent diffusion coefficient (ADC). Combining T2-weighting, high b-value signal and the ADC map has demonstrated as high as 92.4% accuracy in distinguishing between benign and malignant or undetermined uterine neoplasms [10].

Findings and procedure details •

Patient median age of presentation was 63 years-old (17 to 89 years-old).

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The main histological subtypes were leiomyosarcomas with 47% (n: 10), adenosarcomas with 24% (n: 5), endometrial stromal sarcomas with 19% (n: 4) and undifferentiated endometrial sarcomas with 10% (n: 2).

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Clinical manifestations were unspecific, mainly relating to vaginal bleeding, pelvic mass and pelvic pain.

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MRI allowed detailed mass characterization, with 57% (n: 12) of uterine sarcoma presentations revealing an enlarged uterus, 57% (n: 12) demonstrating ill-defined tumour contours and 29% (n:6) being hypervascularized. Necrosis was present in 43% (n: 9) as well as haemorrhagic transformation (29% (n: 6)), translating rapid tumour growth and tissue ischemia. Hypercellularity as observed in DWI was described in 14% (n: 3). Regarding some of the more typical characteristics described in the literature, relating to histological type, in our population 40% (n: 4) of leiomyosarcomas had distant spread (1 case of liver and lung metastases, 1 of lung metastases, 1 to muscular tissue and 1 to peritoneal lining), 50% (n: 3) of endometrial stromal sarcoma cases presented with typical lymphatic and vascular invasion through the myometrium with "bag of worms" appearance and 40% (n: 2) of adenosarcoma cases presented with multiseptated cystic aspect, protruding through the cervical os.

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Conclusion Imaging evaluation by MRI, including diffusion-weighted imaging, has an important role in the assessment of uterine sarcomas, allowing mass characterization as well as adequate staging, consequently contributing to appropriate management.

Personal information Alexandre Gomes Martins Batista th

5 year Radiology Resident José Joaquim Fernandes Hospital, Beja, Portugal. Particular interest in Musculoskeletal Radiology, namely Joint MRI. Contact: [email protected] Teresa Margarida Cunha Department of Radiology Instituto Português de Oncologia de Lisboa Francisco Gentil R. Prof. Lima Basto 1099-023 Lisboa Portugal [email protected]

References 1. D'Angelo A, Prat J. Uterine sarcomas: a review. Gynecol Oncol 2010; 116:131-9. 2. FIGO Committee on Gynecologic Oncology. FIGO staging for uterine sarcomas. Int J Gynaecol Obstet 2009; 106:277.

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3. McCluggage WG. Malignant biphasic uterine tumours: carcinosarcomas or metaplastic carcinomas? J Clin Pathol 2002; 55(5):321-5. 4. Seddon BM, Davda R. Uterine sarcomas - recent progress and future challenges. Eur J Radiol 2011; 78:30-40. 5. Sala E, Rockall AG, Freeman SJ, Mitchell DG, Reinhold C. The added role of MR imaging in treatment stratification of patients with gynecologic malignancies: what the radiologist needs to know. Radiology 2013; 266:717-40. 6. Harlow BL, Weiss NS, Lofton S. The epidemiology of sarcomas of the uterus. J Natl Cancer Inst 1986; 76(3):399-402. 7. Ueda M, Otsuka M, Hatakenaka M, et al. MR imaging findings of uterine endometrial stromal sarcoma: differentiation from endometrial carcinoma. Eur Radiol 2001; 11(1):28-33. 8. Lenhard SM, Untch M, Himsl I, et al. The high-grade endometrial sarcoma: a rare entity. Arch Gynecol Obstet 2006; 274:56-9. 9. Takeuchi M, Matsuzaki K, Yoshida S, et al. Adenosarcoma of the uterus: magnetic resonance imaging characteristics. Clin Imaging 2009; 33:244-47. 10. Thomassin-Naggara I, Dechoux S, Bonneau C, et al. How to differentiate benign from malignant myometrial tumours using MR imaging. Eur Radiol 2013; 23:2306-14.

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