- Case Report
- Open Access
Primary angiosarcoma of the ovary with prominent fibrosis of the ovarian stroma. Case report of an 81-year old patient
https://doi.org/10.1186/1746-1596-6-65
© Bösmüller et al; licensee BioMed Central Ltd. 2011
- Received: 21 April 2011
- Accepted: 14 July 2011
- Published: 14 July 2011
Abstract
Primary angiosarcoma of the ovary (AS) is a rare entity with only 31 reported cases. The majority are pure angiosarcomas, the remainder are associated either with teratomas or conventional epithelial tumors. More than 50% of ovarian AS are disseminated at the time of diagnosis, the minority is detected in stage I. The prognosis of ovarian angiosarcoma in general is poor. Most reports refer to younger individuals, aged from 7 to 46 years, and only 2 case reports could be found for patients older than 64 years. Here we present a very unusual case of angiosarcoma in a 81-year-old patient.
Virtual Slides
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Keywords
- Synovial Sarcoma
- Angiosarcoma
- Virtual Slide
- Mucinous Cystadenoma
- Mature Cystic Teratoma
Background
Survey of all reported cases of ovarian angiosarcoma including histologic type, stage, author, year of publication and citation number
NR | AGE | HISTOLOGY | STAGE | AUTHOR | YEAR | CITATION |
---|---|---|---|---|---|---|
1 | 7 | AS | ? | Evtushenko NT | 1958 | 24 |
2 | 19 | AS | IV | Cunningham MJ | 1994 | 10 |
3 | 19 | AS | III | Davidson B | 2005 | 16 |
4 | 20 | AS | I | Nielsen GP | 1997 | 3 |
5 | 21 | AS | IV | Bouchi J | 1993 | 12 |
6 | 25 | AS | III | Nucci MR | 1998 | 17 |
7 | 25 | AS | III | Lifschitz-Mercer B | 1998 | 22 |
8 | 20-32 * | AS | I | Nielsen GP | 1997 | 3 |
9 | 20-32 * | AS | I | Nielsen GP | 1997 | 3 |
10 | 20-32 * | AS | I | Nielsen GP | 1997 | 3 |
11 | 20-32 * | AS | III | Nielsen GP | 1997 | 3 |
12 | 27 | AS | I | Nucci MR | 1998 | 17 |
13 | 28 | AS | I | Jha S | 2005 | 20 |
14 | 31 | AS | I | Quesenberry CD | 2005 | 21 |
15 | 33 | AS | IV | Nara M | 1996 | 15 |
16 | 35 | AS | IV | Nucci MR | 1998 | 17 |
17 | 38 | AS | IV | Twu NF | 1999 | 11 |
18 | 40 | AS | IV | Platt JS | 1999 | 14 |
19 | 42 | AS | IV | Patel T | 1991 | 13 |
20 | 42 | AS | I | Nucci MR | 1998 | 17 |
21 | 46 | AS | I | Furihata M | 1998 | 19 |
22 | 67 | AS | III | Bradford L | 2009 | 18 |
23 | 81 | AS | I | Bösmüller H | 2011 | |
24 | 20-32 * | Mature cystic teratoma + AS | III | Nielsen GP | 1997 | 3 |
25 | 20-32 * | Mature cystic teratoma + AS | III | Nielsen GP | 1997 | 3 |
26 | 30 | Mature cystic teratoma + AS | III | den Bakker MA | 2006 | 2 |
27 | 32 | Mature cystic teratoma + AS | IV | Contreras AL | 2009 | 1 |
28 | 37 | Mucinous cystadenocarcinoma + AS | I | Jylling AM | 1999 | 5 |
29 | 45 | Borderline serous cystadenocarcinoma + AS | IV | Pillay K | 2001 | 6 |
30 | 77 | Mucinous cystadenoma + AS | III | Ongkasuwan C | 1982 | 4 |
31 | 65 | Ovarian fibroma + AS | I | Cambruzzi E | 2010 | 7 |
Case Presentation
Patient
The 81-year-old patient was admitted to the hospital with abdominal pain and distension. Ultra-sonography and computed tomography revealed a huge cystic mass of the right ovary. There were no signs of distant tumor deposits. The patient underwent hysterectomy and adnexectomy on the right, the left adnexa had been taken out decades ago. Further exploration of the abdomen and all other clinical investigations were without pathological findings, six weeks after laparatomy the patient underwent chemotherapy with 4 cycles of doxyrubicin, and after an follow up of 5 months she is still alive, and there are no signs of recurrence.
Cut surface of the right ovary featuring a centrally located tumor associated cyst.
Transformation from centrally located angiosarcoma high grade (left) to more vasculated and well differentiated tumor areas in the periphery; H&E 40×.
Centrally located angiosarcoma high grade with fusiform tumor component. CD31 staining 400×.
Atypical vascular proliferation in the cortical areas of the ovary. CD31 staining 100×.
The central highly atypical fusiform tumor infiltrate showed intense staining for CD31, reacted weakly for WT1, but had lost expression of CD34. There were nearly no remaining vascular spaces, and we found a Mib-score of 60%. The more angiomatoid proliferation in the periphery did express both, CD31 and CD34, and Ki-67 was expressed only in some of the atypical endothelial cells (Mib-score < 10%). HHV8, epithelial markers (CK7, CAM 5.2, EMA), and smooth muscle actin were negative. Fluorescent in situ hybridisation for SYT-SSX (X; 18) was performed with LSI SYT Dual Colour Break Apart probe (18q11.2, Abbott, North Chicago, Illinois, USA) and was negative. Based on these findings, the patient was diagnosed with primary angiosarcoma of the ovary, high grade.
Discussion
Centrally located high grade angiosarcoma exhibiting atypical mitosis; H&E 200×.
Of 31 reported cases of ovarian angiosarcomas, 23 were pure lesions without coexisting benign or malignant epithelial components. In 5 reports, angiosarcoma was found to be associated with mature cystic teratoma, and in this context it was discussed, whether angiosarcoma is a sarcomatous teratoma, particularly those tumors occurring in younger women[1–3]. In another 3 cases mucinous cystadenoma, mucinous cystadenocarcinoma and borderline serous tumor were coexisting to ovarian AS, rendering the diagnosis adenosarcoma and carcinosarcoma, respectively [4–6], and putting ovarian AS into the context of malignant mesodermal mixed tumor (MMMT) [22]. Angiosarcoma itself may show epitheloid features and can therefore be mistaken for carcinoma or metastasis, and one published case had a predominant reticular growth pattern resembling yolk sac tumor [17].
Conclusions
These examples show that the suspicion of ovarian AS opens a broad range of differential diagnostic considerations. The correct histopathological diagnosis, however, is of importance because prognosis of ovarian AS is uniformly poor. This could be related to the fact that most patients are diagnosed with advanced disease in stage III or IV. Although patients with stage I disease were shown to survive and even become pregnant [3, 20], there are also reports on fatalities of patients with early stage disease.
Consent
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Declarations
Authors’ Affiliations
References
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