Vascular leiomyosarcoma originating from the right ovarian vein: a case report and literature review

Our plain-English summary of Vascular leiomyosarcoma originating from the right ovarian vein: a case report and literature review, by T. Hirano, H. Okumura, S. Maeda, et al (Surgical Case Reports, 2019).

The study

• Authors: T. Hirano, H. Okumura, S. Maeda, M. Shimada, A. Imakiire, K. Makisumi, M. Higashi, S. Natsugoe
• Journal: Surgical Case Reports
• Published: 2019;5(1):120
• Cited by: 10 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Background: Primary leiomyosarcoma (LMS) of vascular origin is a rare lesion, and patients with LMS of vascular origin have poorer prognoses than patients with LMS of other origins. The inferior vena cava is the most commonly affected vessel and accounts for 60% of all vascular cases. However, LMS originating from the ovarian vein is extremely rare, and we are only aware of 15 reported cases. Therefore, we report our experience with a case of LMS originating from the right ovarian vein and review the related literature.

Case Presentation: A 71-year-old Japanese woman with no symptoms was admitted to our hospital because of abnormal findings in a routine abdominal ultrasonography check-up. Contrast-enhanced computed tomography of the abdomen revealed a well-defined, lobulated solid mass with a diameter of 5.5 cm in the right retroperitoneal space. The mass exhibited relatively low uptake during 18F-fluorodeoxyglucose positron emission tomography. Based on these findings, the differential diagnosis included a retroperitoneal tumor, such as a desmoid tumor, leiomyoma, LMS, and malignant mesothelioma. Operative findings confirmed that the mass had originated from the right ovarian vessels, and en bloc excision was performed for the mass and the right ovarian vessels. The final pathological diagnosis was LMS originating from the right ovarian vein, and the surgical resection margins were free from tumor cells. After histological findings confirmed the LMS diagnosis, the patient underwent adjuvant radiation therapy and has not exhibited signs of local recurrence or metastasis in the 6 months after surgery.

Conclusions: We encountered a 71-year-old woman with LMS originating from her right ovarian vein. The prognosis of vascular LMS is generally poor. Therefore, careful follow-up will be required for our patient.

In plain English

This article was a case report with a review of the published literature. It did not study asbestos or any other exposure. Malignant mesothelioma appeared only as one of several possible diagnoses considered before surgery. The patient was a 71-year-old Japanese woman with no symptoms. A routine ultrasound check-up led to scans that found a 5.5 cm solid mass in the right retroperitoneal space, the area at the back of the abdomen. Surgeons found the mass connected to the right ovarian vessels and removed it together with them. Pathologists diagnosed leiomyosarcoma, a cancer of smooth muscle, arising from the right ovarian vein. The edges of the removed tissue were free of tumor cells. She then had radiation therapy, and at 6 months after surgery there were no signs of the tumor returning or spreading. The authors also reviewed 15 earlier reported cases and summarized them together with their own case. In that combined summary, recurrence information was available for 12 cases, and the tumor came back after surgery in 6. The authors concluded that because vascular leiomyosarcoma generally has a poor prognosis, careful follow-up would be required for their patient.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Case report with literature review
• Who was studied: One 71-year-old previously healthy Japanese woman without symptoms, treated at Southern Region Hospital, Makurazaki, Kagoshima, Japan. The authors also reviewed 15 previously reported cases of leiomyosarcoma of the ovarian vein and summarized them together with the present case.
• Exposure: No asbestos or other exposure was studied. Malignant mesothelioma was mentioned only as one possibility in the differential diagnosis before surgery.
• Main results:
  • In the patient, CT showed a well-defined, lobulated solid mass 5.5 cm across in the right retroperitoneal space.
  • In the patient, the mass showed relatively low uptake on FDG-PET, with a maximum standardized uptake value of 2.4.
  • In the patient, the final pathological diagnosis was leiomyosarcoma originating from the right ovarian vein, and the surgical margins were free of tumor cells.
  • The patient received adjuvant radiation therapy (total dose 50.4 Gy in 25 fractions). She showed no signs of local recurrence or metastasis in the 6 months after surgery.
  • The authors pooled the 15 reported cases with their own case. In that pooled summary, the tumor recurred after radical resection in 6 of the 12 cases with recurrence information (50%).
  • Among the cases summarized in the authors' tables, all patients with more than 20 mitoses per 10 high-power fields developed recurrence after radical resection. Table 2 shows 3 of 3 such patients with recurrence, compared with 0 of 4 below 20 (p value 0.03, n = 7).
• The authors' conclusion: The authors concluded that the prognosis of vascular leiomyosarcoma is generally poor, so careful follow-up would be required for their patient.
• Limitations the authors state:
  • In the reviewed cases without recurrence, the median observation period was only 13.5 months. The authors said this is too short to judge prognosis accurately.
  • The analysis of recurrence found no significant risk factors. The authors said this may be due to the small number of cases.
  • The authors said the prognosis of this tumor is obscured by its rarity and by short-term observation data.

Topics

Medical terms in this study, explained in our Lexicon: Differential Diagnosis, Mesothelioma, Surgical Resection, Metastasis, Prognosis.

Related studies

• Hamartoma of mature cardiomyocytes in right atrium: A case report and literature review — X. Zhou, Y. Zhou, et al. (2019)
• Treatment of recurrent isolated right atrial metastatic cavitary mass from breast cancer with radiation therapy: A case report and review of literature — L. Ya'qoub, S. Larson, et al. (2018)
• Malignant epithelioid vascular tumors of the pleura: report of a series and literature review — P.J. Zhang, V.A. Livolsi, et al. (2000)
• Does exposure to asbestos cause ovarian cancer? A systematic literature review and meta-analysis — A. Reid, N. de Klerk, et al. (2011)
• Primary Ovarian Mesothelioma: A Case Series with Electron Microscopy Examination and Review of the Literature — L. Vimercati, D. Cavone, et al. (2021)
• A case of mesothelioma masquerading pre-operatively as ovarian cancer and brief review of the literature — K.L. Hancock, C.M. Clinton, et al. (2016)

Browse more research in Surgical Case Reports, or return to medical research.