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Case Report

Primary Ovarian Epithelioid Angiosarcoma with Extensive Lymph Node Metastases: A Case Report and Literature Review

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DOI:

10.3791/72232

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August 14th, 2026

* These authors contributed equally

In This Article

Summary

Primary ovarian epithelioid angiosarcoma is an exceptionally rare, aggressive vascular malignancy with a challenging preoperative diagnosis. This report describes extensive pelvic and para-aortic lymph node metastases and diffuse intra-abdominal dissemination, confirmed by histopathology and immunohistochemistry. Complete macroscopic cytoreduction was feasible, but prognosis remained poor, highlighting the need for improved therapies worldwide.

Abstract

Primary ovarian epithelioid angiosarcoma (EAS) is an exceptionally rare and highly aggressive vascular malignancy with only isolated cases reported in the literature. Owing to its nonspecific clinical presentation and overlapping radiological features, preoperative diagnosis is extremely challenging and often mimics more common gynecologic malignancies. Consequently, evidence regarding optimal diagnosis and management remains limited.

A 61-year-old postmenopausal woman presented with abdominal pain and a rapidly enlarging pelvic mass. Ultrasonography, computed tomography, and magnetic resonance imaging demonstrated a giant hypervascular pelvic tumor with extensive pelvic and para-aortic lymphadenopathy, initially suggesting uterine sarcoma. Serum CA125 and lactate dehydrogenase levels were elevated. Exploratory laparotomy identified a dominant right ovarian hypervascular tumor with diffuse peritoneal dissemination and bulky pelvic and para-aortic nodal metastases. Comprehensive clinical, radiological, intraoperative, gross pathological, histopathological, and immunohistochemical assessment demonstrated a primary ovarian origin, with no evidence of an extra-ovarian primary angiosarcoma. The patient underwent radical cytoreductive surgery, including total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, appendectomy, resection of visible metastatic lesions, and systematic pelvic and para-aortic lymphadenectomy, achieving complete macroscopic cytoreduction. Histopathological examination demonstrated epithelioid tumor cells with vasoformative differentiation, while immunohistochemistry showed diffuse strong positivity for CD31, CD34, and FLI1, confirming primary ovarian epithelioid angiosarcoma. The patient declined adjuvant chemotherapy, developed rapid postoperative disease progression, and died approximately 2 months after surgery.

This case highlights the marked diagnostic challenges and highly aggressive biological behavior of primary ovarian EAS with extensive pelvic and para-aortic lymph node metastases and diffuse intra-abdominal dissemination. Accurate diagnosis required a comprehensive clinicopathological correlation supported by histopathological and immunohistochemical evaluation. Although complete macroscopic cytoreduction and systematic lymphadenectomy were technically feasible, their therapeutic benefit cannot be inferred from a single case. This report expands the limited literature and emphasizes the need for improved diagnostic strategies, collaborative data collection, and more effective systemic therapies for this rare malignancy.

Introduction

Epithelioid angiosarcoma (EAS) is a rare and highly aggressive malignant vascular neoplasm of mesenchymal origin with endothelial differentiation, accounting for approximately 1%–2% of all soft tissue sarcomas1. Epithelioid angiosarcoma is a distinct histological variant of angiosarcoma characterized by malignant endothelial cells with epithelioid morphology, marked cytologic atypia, and vasoformative differentiation, as recognized in the current World Health Organization (WHO) classification of soft tissue and bone tumors2. Epithelioid angiosarcoma has subsequently been reported in multiple anatomical locations, including the skin, breast, kidney, gastrointestinal tract, lung, and pleura3,4,5,6. Rare presentations have also been described in unusual sites such as the aorta following endovascular repair and the cranial region, where the tumor may manifest as a subdural hematoma secondary to skull involvement7,8.

Angiosarcoma is characterized by aggressive biological behavior, rapid progression, early metastatic dissemination, and poor survival outcomes. Surgical resection remains the cornerstone of treatment for localized and potentially resectable disease because it provides both diagnostic tissue and therapeutic cytoreduction, while histopathological and immunohistochemical evaluation remain essential for definitive diagnosis9,10. Complete macroscopic cytoreduction may be considered in selected patients with resectable advanced disease to achieve maximal tumor debulking and obtain adequate tissue for definitive pathological diagnosis; however, evidence supporting the therapeutic benefit of extensive cytoreduction or systematic lymphadenectomy in primary ovarian epithelioid angiosarcoma remains extremely limited because of the rarity of the disease11,12,13.

Primary ovarian angiosarcoma is an exceptionally uncommon malignancy, with only isolated cases reported in the literature9,10. The epithelioid subtype is particularly rare and is associated with an especially aggressive clinical course. Reported cases have demonstrated heterogeneous presentations, variable treatment strategies, and consistently poor outcomes11,12,13,14,15,16,17,18,19,20,21,22. Because clinical manifestations and imaging findings are nonspecific, ovarian EAS is frequently misdiagnosed preoperatively as epithelial ovarian carcinoma, metastatic disease, or uterine sarcoma9,10,19,22. In addition, metastatic angiosarcoma involving the ovary should be considered in the differential diagnosis, making comprehensive clinicoradiological, intraoperative, histopathological, and immunohistochemical correlation essential for establishing a primary ovarian origin. This diagnostic uncertainty complicates surgical planning and intraoperative decision-making, particularly regarding the extent of cytoreductive surgery and the management of clinically suspicious lymph nodes in advanced disease.

The overall goal of the present report is to expand the limited clinicopathological evidence regarding primary ovarian EAS and to provide additional insight into its diagnostic challenges, metastatic behavior, surgical management, and clinical outcomes. We present a rare case of primary ovarian epithelioid angiosarcoma with extensive pelvic and para-aortic lymph node metastases, initially misdiagnosed as uterine sarcoma, together with a review of previously published cases. The diagnosis of primary ovarian epithelioid angiosarcoma was supported by the presence of a dominant right ovarian mass, the gross and microscopic relationship of the tumor to ovarian tissue, the distribution of metastatic disease, and comprehensive clinical and radiological evaluation demonstrating no evidence of a primary angiosarcoma at another anatomical site. The rationale for presenting this case lies in the extremely rare occurrence of widespread nodal metastases and diffuse intra-abdominal dissemination, features that are scarcely documented in the current literature. Although complete macroscopic cytoreduction with systematic pelvic and para-aortic lymphadenectomy was technically feasible in this patient, the present report does not imply a therapeutic or survival benefit of this approach; rather, it illustrates its role in achieving complete gross resection, accurate pathological assessment, and documentation of the extent of disease. By contextualizing this case within the wider body of published literature, we aim to assist clinicians and surgeons in recognizing clinical scenarios in which ovarian EAS should be considered in the differential diagnosis of rapidly enlarging hypervascular pelvic tumors with extensive nodal involvement.

Case Presentation

A 61-year-old postmenopausal woman presented to the Department of Gynecology at Gansu Provincial Maternal and Child Health Hospital in March 2023 with a 2-week history of intermittent abdominal pain and progressive abdominal enlargement. Physical examination revealed a large abdominopelvic mass extending approximately 10 cm above the umbilicus. Laboratory investigations demonstrated elevated serum CA125 (156.1 U/mL) and lactate dehydrogenase (257 U/L) levels, while other routinely assessed gynecologic tumor markers were within normal limits.

Pelvic ultrasonography, contrast-enhanced computed tomography, and magnetic resonance imaging demonstrated a large hypervascular right adnexal mass with extensive pelvic and para-aortic lymphadenopathy, raising suspicion for an advanced gynecologic malignancy, initially considered to represent uterine sarcoma. Comprehensive preoperative imaging demonstrated no evidence of a primary vascular malignancy outside the pelvis.

The patient underwent exploratory laparotomy with the goal of complete macroscopic cytoreduction and definitive diagnosis. Intraoperatively, a dominant right ovarian hypervascular tumor with diffuse peritoneal dissemination and bulky pelvic and para-aortic lymph node involvement was identified, and radical cytoreductive surgery was performed. The final diagnosis of primary ovarian epithelioid angiosarcoma was established by histopathological and immunohistochemical evaluation. Despite an initially uncomplicated postoperative recovery, the patient declined adjuvant chemotherapy, developed rapidly progressive metastatic disease, and died approximately 2 months after surgery. The overall clinical course is summarized in Table 1.

Diagnosis, Assessment, and Plan

The patient presented with a rapidly enlarging abdominopelvic mass, abdominal pain, elevated serum CA125 and lactate dehydrogenase levels, and imaging findings suggestive of an advanced gynecologic malignancy. Pelvic ultrasonography, contrast-enhanced computed tomography, and magnetic resonance imaging demonstrated a large hypervascular right adnexal mass with extensive pelvic and para-aortic lymphadenopathy. Comprehensive preoperative imaging demonstrated no evidence of a primary vascular malignancy outside the pelvis.

The differential diagnosis included uterine sarcoma, epithelial ovarian carcinoma, metastatic carcinoma, and other primary gynecologic sarcomas, as well as less common vascular and epithelioid neoplasms. Because the radiological findings were nonspecific, a definitive preoperative diagnosis could not be established. Given the extensive but potentially resectable disease, exploratory laparotomy was undertaken with the goals of complete macroscopic cytoreduction and definitive pathological diagnosis. Intraoperative frozen section examination was not performed because the operative findings indicated that immediate cytoreductive surgery would be required regardless of the pathological impression.

The patient underwent total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, appendectomy, resection of visible metastatic lesions, and systematic pelvic and para-aortic lymphadenectomy, achieving complete macroscopic cytoreduction. Histopathological and immunohistochemical evaluation confirmed the diagnosis of primary ovarian epithelioid angiosarcoma. Adjuvant chemotherapy was recommended because of the aggressive nature of the disease and extensive metastatic involvement; however, the patient declined further treatment and subsequently developed rapidly progressive disease.

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Protocol

The surgical management was performed in accordance with institutional and national ethical guidelines. The case report and surgical protocol were reviewed and approved by the Institutional Ethics Committee of Gansu Provincial Maternal and Child Health Hospital. Written informed consent for treatment and publication of the case details and accompanying images was obtained from the patient prior to their passing.

1. Preoperative clinical evaluation

  1. Initial clinical assessment
    1. The patient underwent a detailed clinical evaluation following presentation with intermittent abdominal pain and progressive abdominal enlargement. A comprehensive medical history, including gynecologic, surgical, cardiovascular, family, occupational, and environmental history, was obtained.
    2. Physical examination was performed to assess abdominal distension, palpable mass characteristics, and evidence of metastatic disease.
    3. Clinical assessment also included evaluation for clinical features suggestive of an extra-ovarian primary malignancy. No clinical findings indicated a primary vascular neoplasm outside the female genital tract, and subsequent radiological and intraoperative assessments likewise demonstrated no evidence of another primary angiosarcoma.
  2. Laboratory investigations
    1. Baseline hematological and biochemical investigations were performed, including complete blood count, liver and renal function tests, serum electrolytes, and coagulation profile.
    2. Tumor marker evaluation included serum CA125, lactate dehydrogenase (LDH), CA19-9, CA15-3, carcinoembryonic antigen (CEA), and human epididymis protein 4 (HE4) levels to aid differential diagnosis of gynecologic malignancy.
      ​NOTE: Although serum CA125 and LDH levels were elevated, these biomarkers were interpreted as nonspecific indicators of tumor burden, peritoneal involvement, and extensive intra-abdominal disease rather than diagnostic biomarkers of primary ovarian epithelioid angiosarcoma.
  3. Radiological assessment
    1. Pelvic ultrasonography was initially performed to characterize the pelvic mass and evaluate internal vascularity. Representative ultrasonographic findings are shown in Supplementary Figure 1.
    2. Contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) of the abdomen and pelvis were subsequently obtained to determine tumor origin, local invasion, lymph node involvement, and intra-abdominal dissemination. Contrast-enhanced CT demonstrated a hypervascular right adnexal mass with enlarged pelvic and para-aortic lymph nodes (Supplementary Figure 2).
      NOTE: All imaging studies were independently reviewed by radiologists experienced in gynecologic oncology imaging.
    3. Ultrasonography demonstrated maximal tumor dimensions of approximately 31 cm × 28 cm, whereas MRI demonstrated a lesion measuring 19.5 cm × 20.1 cm in the axial plane. These differences reflected variation in imaging modality, measurement plane, and assessment technique.
    4. Comprehensive cross-sectional imaging demonstrated no evidence of a primary angiosarcoma or other vascular malignancy at another anatomical site, supporting a primary ovarian origin.

2. Preoperative diagnostic planning

  1. Differential diagnostic evaluation
    1. Based on the clinical presentation, laboratory investigations, and multimodal imaging, the differential diagnosis included uterine sarcoma, epithelial ovarian carcinoma, metastatic carcinoma, and other primary gynecologic sarcomas.
    2. Less common considerations included metastatic angiosarcoma, epithelioid sarcoma, malignant melanoma, epithelioid hemangioendothelioma, Müllerian neoplasms, sex cord-stromal tumors, and other epithelioid vascular neoplasms.
    3. Comprehensive preoperative imaging demonstrated no evidence of a primary vascular malignancy outside the pelvis.
      NOTE: Because the radiological findings were nonspecific and overlapped with those of several gynecologic malignancies, a definitive preoperative diagnosis could not be established.
  2. Surgical decision-making
    1. The patient's case was reviewed by a multidisciplinary gynecologic oncology team. Based on the clinical findings, imaging studies, and the presence of advanced but potentially resectable disease, exploratory laparotomy was recommended.
    2. The primary objectives of surgery were to establish a definitive diagnosis through pathological evaluation and to achieve complete macroscopic cytoreduction when technically feasible.

3. Operative procedure

  1. Exploratory laparotomy
    1. Under general anesthesia, an exploratory laparotomy was performed through a midline abdominal incision. The abdominal cavity was systematically inspected to determine the tumor origin and extent of metastatic dissemination.
    2. Systematic exploration included evaluation of the peritoneal cavity, liver surface, diaphragm, omentum, bowel, mesentery, pelvic organs, retroperitoneum, and para-aortic region.
    3. Particular attention was directed toward identifying the dominant tumor site and excluding a primary angiosarcoma or other vascular malignancy at another anatomical location. Representative preoperative radiological and intraoperative findings are shown in Figure 1.
    4. No evidence of a primary angiosarcoma outside the ovary was identified during systematic intra-abdominal exploration.
      NOTE: Intraoperative frozen section examination was not performed because the extensive intra-abdominal dissemination, bulky pelvic and para-aortic nodal disease, and technically resectable tumor burden warranted immediate radical cytoreductive surgery to achieve complete macroscopic resection and obtain adequate tissue for definitive histopathological diagnosis.
  2. Intraoperative tumor assessment
    1. The primary tumor was identified as arising from the right ovary and demonstrated marked vascularity with nodular surface irregularity. The dominant tumor was centered within the right ovary and maintained gross continuity with ovarian tissue.
    2. Intraoperative evaluation documented metastatic involvement of the left ovary, uterine serosa, cervix, pelvic peritoneum, sigmoid mesentery, appendix, and pelvic and para-aortic lymph nodes. The extent of metastatic dissemination was systematically documented before resection, and complete macroscopic cytoreduction was considered technically achievable.
  3. Radical cytoreductive surgery
    1. Radical cytoreductive surgery was subsequently performed and included total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, appendectomy, resection of all visible metastatic lesions, and systematic pelvic and para-aortic lymphadenectomy.
    2. Pelvic lymphadenectomy included the bilateral external iliac, internal iliac, obturator, and common iliac nodal basins, whereas para-aortic lymphadenectomy extended from the aortic bifurcation to the level of the inferior mesenteric artery.
    3. Surgical resection was continued until complete macroscopic cytoreduction (CC-0) was achieved. Complete macroscopic cytoreduction was confirmed by systematic inspection and palpation of the abdominal cavity at the completion of surgery, with no visible residual tumor remaining.
      NOTE: The objective of systematic lymphadenectomy in this patient was to facilitate accurate pathological staging and resection of bulky metastatic lymph nodes rather than to imply a proven therapeutic or survival benefit.
  4. Intraoperative monitoring and management
    1. Estimated blood loss and operative duration were recorded throughout the procedure. Continuous hemodynamic monitoring, including electrocardiography, arterial blood pressure, pulse oximetry, end-tidal carbon dioxide, and urine output, was maintained according to standard anesthetic practice.
    2. Intravenous crystalloid fluid replacement was administered as clinically indicated.

4. Histopathological and immunohistochemical evaluation

  1. Tissue processing
    1. Resected surgical specimens were fixed in 10% neutral-buffered formalin for 24–48 h, processed using standard automated tissue-processing protocols, and embedded in paraffin according to routine histopathological procedures.
    2. Representative tissue sections were obtained from the primary ovarian tumor, bilateral adnexa, uterus, cervix, pelvic peritoneum, sigmoid mesentery, appendix, omentum, and all resected lymph nodes.
    3. Formalin-fixed, paraffin-embedded (FFPE) tissue blocks were sectioned at approximately 4 µm thickness, and hematoxylin-eosin (HE) staining was performed for microscopic evaluation.
    4. Gross pathological assessment included measurement of tumor dimensions and weight, evaluation of ovarian surface involvement, hemorrhage, tumor necrosis, surgical margin status, lymphovascular invasion, and regional lymph node metastases.
  2. Histopathological examination
    1. Microscopic examination assessed tumor architecture, cytologic atypia, mitotic activity, vasoformative differentiation, vascular channel formation, and metastatic involvement of the resected tissues and lymph nodes.
    2. Histopathological evaluation additionally documented irregular anastomosing vascular channels, intracytoplasmic erythrocyte-containing lumina, tumor necrosis, lymphovascular invasion, ovarian surface involvement, and surgical margin status.
    3. Mitotic activity was quantified by counting mitotic figures in 10 consecutive high-power fields (HPFs).
    4. Regional lymph nodes were examined individually to determine the number of metastatic lymph nodes, the size of metastatic deposits, and the presence or absence of extranodal extension.
    5. Pathological staging was assigned according to the FIGO ovarian carcinoma staging framework and the AJCC TNM Classification (8th edition).
  3. Immunohistochemical analysis
    1. Immunohistochemical staining was performed using endothelial markers, including CD31, CD34, and FLI1. Additional markers, including cytokeratin (AE1/AE3), epithelial membrane antigen (EMA), ERG, D2-40, PAX8, WT1, HMB45, TFE3, CD10, vimentin, SMARCA4 (BRG1), BCOR, Ki-67, and p53, were evaluated to exclude alternative diagnoses and confirm endothelial differentiation.
      1. Staining was performed on formalin-fixed, paraffin-embedded tissue sections using validated clinical antibodies according to the manufacturer's recommendations following heat-induced antigen retrieval.
      2. Appropriate external positive and negative tissue controls were included in each staining run. Membranous staining was evaluated for CD31 and CD34; nuclear staining for FLI1, ERG, PAX8, WT1, TFE3, SMARCA4, BCOR, Ki-67, and p53; and cytoplasmic and/or membranous staining for cytokeratin (AE1/AE3), EMA, D2-40, HMB45, CD10, and vimentin, according to the expected localization of each marker.
      3. Immunoreactivity was independently assessed by two experienced pathologists. Staining intensity was graded as 0 (negative), 1+ (weak), 2+ (moderate), or 3+ (strong), and the percentage of positive tumor cells was recorded.
        ​NOTE:. Detailed information regarding antibody clones, manufacturers, catalog numbers (where available), working dilutions, antigen retrieval conditions, detection system, and staining platform is provided in the accompanying Table of Materials.

5. Postoperative management and follow-up

  1. Immediate postoperative care
    1. Following surgery, the patient underwent routine postoperative monitoring, including assessment of vital signs, abdominal examination, pain control, fluid balance, and laboratory investigations.
    2. Serial complete blood count, serum biochemistry, and postoperative hemoglobin measurements were performed according to institutional postoperative protocols.
    3. Serum CA125 and lactate dehydrogenase (LDH) levels were reassessed during postoperative follow-up.
  2. Adjuvant treatment planning
    1. Adjuvant systemic chemotherapy was recommended because of the extensive metastatic disease and the highly aggressive biological behavior of the primary ovarian epithelioid angiosarcoma.
    2. Following multidisciplinary gynecologic oncology review of the final histopathological diagnosis, extensive pelvic and para-aortic nodal metastases, and advanced disease stage, postoperative systemic chemotherapy was advised.
    3. Available treatment options, anticipated benefits, potential adverse effects, and the poor prognosis associated with this malignancy were discussed comprehensively with the patient and her family.
      ​NOTE: Despite detailed counseling, the patient declined further systemic oncologic treatment.
  3. Clinical outcome assessment
    1. Clinical follow-up included evaluation for postoperative complications, biochemical response, and evidence of disease progression.
    2. Follow-up assessment comprised serial clinical examination, laboratory investigations, and radiological evaluation when clinically indicated.
    3. Progressive intra-abdominal metastatic disease was confirmed during follow-up based on clinical deterioration and radiological findings.

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Results

Following appropriate preoperative evaluation and exploratory laparotomy, a dominant hypervascular tumor arising from the right ovary was identified intraoperatively. Extensive metastatic dissemination involving the contralateral ovary, uterine serosa, cervix, pelvic peritoneum, sigmoid mesentery, appendix, and pelvic and para-aortic lymph nodes was observed. Figure 1 illustrates the preoperative radiological findings and intraoperative surgical findings. Radical cytoreductive surgery, inclu...

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Discussion

Primary ovarian angiosarcoma is an exceptionally rare and highly aggressive malignant neoplasm of vascular endothelial origin. The largest clinicopathological analysis was reported by Nielsen et al.11, who described seven ovarian angiosarcoma cases and emphasized the aggressive biological behavior and poor prognosis associated with this entity. Subsequent reports11,12,13,14

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Disclosures

The authors declare that they have no competing interests.

Acknowledgements

This work was partially supported by the Natural Science Foundation of Gansu Province (Grant No. 24JRRA621) and the Natural Science Foundation of Gansu Province (Grant No. 22JR5RA718) and the General Project of Scientific Research Fund, Gansu Provincial Maternity and Child-care Hospital (Gansu Provincial Central Hospital) (Grant No.GMCCH2025-3-2)

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Materials

List of materials used in this article
NameCompanyCatalog NumberComments
10% Neutral Buffered FormalinSigma-Aldrich (Merck)HT501128Tissue fixation
Automated Tissue ProcessorLeica BiosystemsASP300 STissue processing
Autostainer Link 48Agilent (Dako)Autostainer Link 48Immunohistochemistry
BCOR antibodySanta Cruz Biotechnologysc-5145761:100 (dilution)
CD10 antibodyLeica BiosystemsPA02701:100 (dilution)
CD31 antibody (JC70A)Agilent/DakoM08231:100 (dilution)
CD34 antibody (QBEnd/10)Agilent/DakoM71651:100 (dilution)
cellSens StandardOlympuscellSensImage analysis
CK AE1/AE3Agilent/DakoM35151:200 (dilution)
CT ScannerSiemens HealthineersSOMATOMContrast CT
D2-40 antibodyAgilent/DakoM36191:100 (dilution)
DAB ChromogenAgilent (Dako)K3468Chromogen
DP74 CameraOlympusDP74Image acquisition
EMA antibody (E29)Agilent/DakoM06131:200 (dilution)
EnVision FLEX HRP Detection KitAgilent (Dako)K8002Polymer HRP detection
ERG antibody (EP111)Cell Marque280M1:100 (dilution)
FLI1 antibody (MRQ-1)Cell Marque249M1:100 (dilution)
HMB45 antibodyAgilent/DakoM06341:100 (dilution)
IBM SPSS Statistics v26IBMv26Statistical analysis
Ki-67 antibodyAgilent/DakoM72401:100 (dilution)
Mayer's HematoxylinAgilent (Dako)S3309Counterstain
Microtome RM2235Leica BiosystemsRM2235Cut 4 μm FFPE sections
MRI ScannerSiemens HealthineersMAGNETOMPreoperative MRI
Olympus BX53 MicroscopeOlympusBX53Histopathology
p53 antibodyAgilent/DakoM70011:100 (dilution)
Paraffin Embedding MediumLeica Biosystems39601095Paraffin embedding
PAX8 antibodyCell Marque363M1:100 (dilution)
Positively Charged Microscope SlidesThermo Fisher ScientificJ1800AMNZMount tissue sections
SMARCA4 (BRG1) antibodyAbcamab1106411:100 (dilution)
TFE3 antibodyCell Marque367M1:100 (dilution)
Ultrasound SystemGE HealthcareLOGIQ E9Color Doppler ultrasound
Vimentin antibodyAgilent/DakoM07251:200 (dilution)
Water BathLeica BiosystemsHI1210Section flotation
WT1 antibodyAgilent/DakoM35611:100 (dilution)

References

  1. Khalil MF, Thomas A, Aassad A, Rubin M, Taub RN. Epithelioid angiosarcoma of the small intestine after occupational exposure to radiation and polyvinyl chloride: a case report and review of the literature. Sarcoma. 2005;9(3–4):161–164.
  2. WHO Classification of Tumours Editorial Board. Soft Tissue and Bone Tumours. 5th ed. International Agency for Research on Cancer (IARC); Lyon; 2020.
  3. Fariña MC, et al. Epithelioid angiosarcoma of the breast involving the skin: a highly aggressive neoplasm readily mistaken for mammary carcinoma. J Cutan Pathol. 2003;30(2):152–156.
  4. Singh C, Xie L, Schmechel SC, Manivel JC, Pambuccian SE. Epithelioid angiosarcoma of the kidney: a diagnostic dilemma in fine-needle aspiration cytology. Diagn Cytopathol. 2012;40(Suppl 2):E131–E139.
  5. Zhou N, et al. Primary gastric epithelioid angiosarcoma: a case report of diagnostic biopsy pitfall. Medicine (Baltimore). 2024;103(40):e39719.
  6. Yang P, Wu Q, Zhou Y, Li Y. Primary epithelioid angiosarcoma of the jejunal mesentery causing abdominal bleeding: a case report and literature review. Onco Targets Ther. 2024;17:327–338.
  7. Zeng A, South A, Slevin M, Lim K. Persistent pain and fever unmasking aortic angiosarcoma at the site of previous endovascular aortic repair. BMJ Case Rep. 2025;18(1):e263591.
  8. Yamada Y, et al. Subdural hematoma caused by epithelioid angiosarcoma originating from the skull. Head Neck Pathol. 2013;7(2):159–162.
  9. Ye H, et al. Primary ovarian angiosarcoma: a rare and recognizable ovarian tumor. J Ovarian Res. 2021;14(1):21.
  10. Bösmüller H, et al. Primary angiosarcoma of the ovary with prominent fibrosis of the ovarian stroma. Case report of an 81-year-old patient. Diagn Pathol. 2011;6:65.
  11. Nielsen GP, Young RH, Prat J, Scully RE. Primary angiosarcoma of the ovary: a report of seven cases and review of the literature. Int J Gynecol Pathol. 1997;16(4):378–382.
  12. Furihata M, et al. Primary ovarian angiosarcoma: a case report and literature review. Pathol Int. 1998;48(12):967–973.
  13. Platt JS, Rogers SJ, Flynn EA, Taylor RR. Primary angiosarcoma of the ovary: a case report and review of the literature. Gynecol Oncol. 1999;73(3):443–446.
  14. Quesenberry CD, Li C, Chen AH, Zweizig SL, Ball HG 3rd. Primary angiosarcoma of the ovary: a case report of Stage I disease. Gynecol Oncol. 2005;99(1):218–221.
  15. Davidson B, Abeler VM. Primary ovarian angiosarcoma presenting as malignant cells in ascites: case report and review of the literature. Diagn Cytopathol. 2005;32(5):307–309.
  16. Vavilis D, et al. Primary ovarian angiosarcoma—review of the literature and report of a case with coexisting chylothorax. Eur J Gynaecol Oncol. 2007;28(4):287–289.
  17. Bradford L, Swartz K, Rose S. Primary angiosarcoma of the ovary complicated by hemoperitoneum: a case report and review of the literature. Arch Gynecol Obstet. 2010;281(1):145–150.
  18. Iljazović E, et al. Angiosarcoma of the ovary in an 11-year-old girl: case report and review of the literature. Bosn J Basic Med Sci. 2011;11(2):132–136.
  19. Yaqoob N, et al. Ovarian angiosarcoma: a case report and review of the literature. J Med Case Rep. 2014;8:47.
  20. Wu PC, Yue CT, Huang SC. Complete response after MAID treatment for advanced primary ovarian angiosarcoma: case report and literature review. Eur J Gynaecol Oncol. 2014;35(3):318–321.
  21. Darré T, et al. Primary ovarian angiosarcoma in a 12-year-old girl: a case report of an exceptional localization in a context of limited resources country. BMC Clin Pathol. 2017;17:16.
  22. Zhou Y, Sun YW, Liu XY, Shen DH. Primary ovarian angiosarcoma: two case reports and review of literature. World J Clin Cases. 2023;11(21):5122–5128.
  23. Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ. Angiosarcoma. Lancet Oncol. 2010;11:983–991.
  24. Young RJ, Woll PJ. Anti-angiogenic therapies for the treatment of angiosarcoma: a clinical update. Memo. 2017;10(4):190–193.
  25. Shon W, Billings SD. Epithelioid vascular tumors: a review. Adv Anat Pathol. 2019;26(3):186–197.
  26. Tai CM, et al. Primary gastric angiosarcoma presenting as an asymptomatic gastric submucosal tumor. J Formos Med Assoc. 2007;106(11):961–964.
  27. Buehler D, et al. Angiosarcoma outcomes and prognostic factors: a 25-year single-institution experience. Am J Clin Oncol. 2014;37(5):473–479.
  28. Fayette J, et al. Angiosarcomas: a heterogeneous group of sarcomas with site-specific behavior. Ann Oncol. 2007;18(12):2030–2036.
  29. Wang X, et al. Characteristics and outcomes of primary pleural angiosarcoma: a retrospective study of 43 cases. Medicine (Baltimore). 2022;101(6):e28785.
  30. Wu N, Hong SK, Huang WF. Unexpected cause of anemia: primary small intestinal angiosarcoma. J Gastrointest Surg. 2023;27(3):633–635.
  31. Perez-Atayde AR, Achenbach H, Lack EE. High-grade epithelioid angiosarcoma of the scalp: an immunohistochemical and ultrastructural study. Am J Dermatopathol. 1986;8(5):411–418.
  32. Miettinen M, et al. ERG transcription factor as an immunohistochemical marker for vascular endothelial tumors and prostatic carcinoma. Am J Surg Pathol. 2011;35(3):432–441.
  33. Cao J, Wang J, He C, Fang M. Angiosarcoma: a review of diagnosis and current treatment. Am J Cancer Res. 2019;9(11):2303–2313.
  34. Zhu C, Yang N, Yao J, Du X. Primary pleural epithelioid angiosarcoma with lung and bone metastases: a case report. Case Rep Oncol. 2024;17(1):101–106.
  35. Fukunaga M. Expression of D2-40 in lymphatic endothelium and vascular tumors. Histopathology. 2005;46(4):396–402.
  36. Yu JH, Cao LL, Qian J. Multiple epithelioid angiosarcoma of stomach and small intestine with lymph node metastases: a case report. Medicine (Baltimore). 2023;102(25):e34024.
  37. Raaf HN, Raaf JH. Sarcomas related to the heart and vasculature. Semin Surg Oncol. 1994;10(5):374–382.
  38. Nai Q, et al. Primary small intestinal angiosarcoma: epidemiology, diagnosis, and treatment. J Clin Med Res. 2018;10(4):294–301.
  39. Tamura K, et al. Endoscopic diagnosis of small intestinal angiosarcoma: a case report. DEN Open. 2021;2(1):e24.
  40. Ryu DY, et al. Primary angiosarcoma of small intestine presenting as gastrointestinal bleeding. Korean J Gastroenterol. 2005;46(5):404–408.
  41. Castro EC, et al. Primary mesenteric angiosarcoma in a child: a case report. Pediatr Dev Pathol. 2008;11(6):482–486.
  42. Young RJ, et al. First-line anthracycline-based chemotherapy for angiosarcoma: pooled EORTC analysis. Eur J Cancer. 2014;50(18):3178–3186.
  43. Goerdt LV, Schneider SW, Booken N. Cutaneous angiosarcomas: molecular pathogenesis and therapeutic approaches. J Dtsch Dermatol Ges. 2022;20(4):429–443.
  44. Sun X, He L, Wang S. Risk factors for pelvic lymph node metastasis in cervical cancer: a retrospective analysis of 186 patients. Front Oncol. 2025;15:1525946.
  45. Sun X, et al. Preoperative malnutrition predicts poor early immune recovery following gynecologic cancer surgery: a retrospective cohort study and risk nomogram development. Front Immunol. 2025;16:1681762.
  46. Ray-Coquard IL, et al. Paclitaxel with or without bevacizumab in advanced angiosarcoma: a randomized phase II trial. J Clin Oncol. 2015;33:2797–2802.
  47. Yao F, He L, Sun X. Predictive factors of chemotherapy-induced nausea and vomiting in elderly patients with gynecological cancer undergoing paclitaxel and carboplatin therapy: a retrospective study. Oncol Lett. 2025;29(4):167.
  48. Zhang SL, Liang L, Ji Y, Wang Z, Zhou Y. Everolimus in recurrent epithelioid angiosarcoma: case reports and literature review. Oncotarget. 2017;8(55):95023–95029.

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Cytoreductive SurgeryImmunohistochemical EvaluationHistopathological AssessmentPelvic LymphadenopathyPara-Aortic LymphadenopathyVascular MalignancyCD31 MarkerOvarian Tumor